Boston University Vasculitis Center

Boston University Rheumatology Section  Rheumatology faculty have achieved widespread national recognition for both their research programs and their clinical expertise. There are currently active clinical trials in vasculitis and section investigators are participating in NIH-sponsored clinical trials in vasculitis.

Buerger's Disease

Buerger's Disease is vasculitis causing insufficient blood flow to the hands and feet, producing numbness, tingling and ultimately skin and gangrene.  The classic Buerger's patient is a male, between the ages of 20 and 40, although there is a higher percentage of women and people over the age of 50 being diagnosed.   Buerger's disease is most common in the Orient, Southeast Asia, India and the Middle East, but appears to be rare among African-Americans.

Diagnosis: Angiograms of the extremities is helpful in making the diagnosis. Skin biopsies are not recommended due to the concern that the biopsy site will not heal.

Treatment: Buerger's disease is associated with smoking and tobacco produces. Patients must stop smoking immediately. Abstinence from these products is the only way to treat this disease. Although anti-inflammatory agents (steroids) have proven beneficial in other vasculitides, they are not beneficial in treating Buerger's.

Central Nervous System (CNS)

Isolated central nervous system vasculitis also known as primary angiitis of the central nervous system (CNS), is a condition where the body's immune system attacks arteries of the brain, causing inflammation (swelling).

The cause of CNS is unknown. Researchers believe that some sort of event, like an infection, may trigger an inappropriate immune response directed mistakenly to the small and medium sized arteries of the brain.

Among the reported cases of CNS, there are more males who are diagnosed with the disease, and although most get the disease in their 40s or 50s, children as young as 3 and adults as old as 78 have been diagnosed with CNS.

The most common symptoms of CNS are: confusion, headache, personality changes, and muscle weakness/paralysis similar to what happens when someone suffers a stroke. Other symptoms include seizures, bleeding in the head, coma, and vision loss. Symptoms usually occur over the course of several months, but can also occur very quickly.

Nonspecific laboratory abnormalities have been noted among patients with CNS. The diagnosis of CNS can be done in different ways. The principal form of diagnosis remains angiography (x-rays of arteries), but magnetic resonance imaging (MRI) and CT scanning have also been utilized. None of these alone can be diagnostic of CNS. A common concern is that angiography in a patient with possible cerebral vessel inflammation is unsafe, and may result in complications. However a study of 125 cerebral angiograms, found no greater risk of complications in patients with proven CNS vasculitis than in those with normal angiograms. A lumbar puncture may also be performed to look for abnormalities in the cerebrospinal fluid.

The symptoms, signs, or findings among patients with CNS may "mimic" a number of disorders including other primary CNS vasculitides, systemic vasculitis, CNS infections or drug-induced disorders by methamphetamine, cocaine, "crack", and ephedrine abuse.

Early reports of patients with cerebral vasculitis suggested a less optimistic prognosis but more recent experience with immunosuppressive therapy has resulted in encouraging outcomes.

If the diagnosis of CNS is confirmed by biopsy, treatment with high doses of glucocorticoids (steroids) is warranted. Cytotoxic agents such as cyclophosphamide (Cytoxan) can be added depending on the clinical severity of the vasculitis.

Side-effects of high-dose glucocorticoids should be anticipated such as glucose intolerance or frank diabetes mellitus (condition of abnormal glucose levels in blood). Use of calcium supplements, vitamin D, hormone replacement therapy, and bisphosphonates can be taken to help protect the body from bone mineral loss. Antibiotics can also be taken to help prevent infection due to the use of immunosuppressants.

Churg Strauss Syndrome (CSS)

Churg Strauss syndrome (CSS), also called allergic granulomatosis and angiitis, is a disorder characterized by allergic rhinitis (inflammation of the nose), asthma, and an alteration in blood counts called eosinophilia. The organs involved are usually the lung and the skin although any organ system can be affected including the cardiovascular (heart), gastrointestinal (stomach), kidneys and the central nervous system.

View all resources for Churg Strauss syndrome.

Cryoglobulinemia

Cryoglobulinemia is the presence of abnormal proteins that are occasionally found in the blood of people with some forms of autoimmune diseases, multiple myeloma, leukemia, and certain forms of pneumonia. The proteins cause the blood to gel at low temperatures causing tissue necrosis.

Causes:  Type 1 cryoglobulinemia is often associated with lymphoma.  Type 2 cryoglobulinemia is often associated with hepatitis C infection.  Drug usage is a prime risk factor for patients with cryoglobulinemia.  Hepatitis C is acquired by injection drug use (needle-sharing), tainted blood products, and (probably rarely), sexual transmission.

Symptoms may include a rash on the lower limbs, arthritis, nerve damage and tissue necrosis of affected areas.

Treatment depends on the type of cryoglobulin, underlying disease, and severity of symptoms. Cryoglobulinemia with severe hyperviscosity syndrome requires plasmapheresis and chemotherapy of the underlying malignancy. Some patients with cryoglobulinemia suffer from mild, recurrent crops of lower extremity purpura that require no specific therapy. The most effective treatment for cryoglobulinemia associated with hepatitis C has not yet been determined. Treating the hepatitis may be an effective therapy for this type of vasculitis. Brief use of prednisone followed by 6 months of interferon alfa has produced clinical and liver function test improvement, but relapse of liver disease and vasculitis often occurs when interferon alfa is stopped.

6.06

Giant Cell Arteritis (GCA)

Giant cell (temporal) arteritis (GCA) is a vasculitis of large and medium size vessels. It may be generalized but vessel inflammation most frequently involves vessels in the scalp and head, especially the arteries over the temples. The disease is called temporal arteritis because the temporal arteries, which course along the sides of the head just in front of the ears (to the temples) often become inflamed. Women, Caucasians, and individuals over 50 years of age are most commonly affected by GCA.

The onset of the symptoms in GCA tends to be gradual and includes low grade fever, fatigue, weakness and weight loss. A new headache, mild or severe, occurs in at least two-thirds of patients with the pain tending to be located over the sides of the head in front of the ears but may be frontal or other located. Nearly one-half of patients suffer from pain in the jaw after chewing (called jaw claudication). Impaired vision is often an early manifestation of the disease. Permanent partial or complete loss of vision in one or both eyes has been observed in 15-20 % of patients. It is rare for patients to become completely blind in both eyes.

Polymyalgia rheumatica (PMR), which is characterized by pain in the shoulders and hips, is closely linked to GCA, occurring in about 40-50 % of patients.

A laboratory abnormality seen in most patients with GCA is a very high erythrocyte sedimentation rate (ESR). The ESR measures how fast a patient's red blood cells settle when placed in a small tube. Anemia or low red blood cell count and microscopic hematuria (blood in the urine) may be found but renal (kidney) impairment is unlikely to be due to GCA. Other tests are occasionally abnormal with non specific meaning.

Temporal artery biopsy is suggested in all cases of suspected GCA. Even though the diagnosis may appear "classic" a temporal artery biopsy is still recommended. The biopsy is of low risk, causes very little pain, and often leaves little or no scar. After the use of a topical numbing medication (the same one used by a dentist), the doctor can remove a small part of the temporal artery from under the scalp in order to examine it under the microscope.

Other ways to diagnose GCA include: ultrasonography, angiographic examination, computerized topographic scanning and magnetic resonance angiography, high resolution magnetic resonance imaging and position emission tomography (PET).

Glucocorticoid treatment should be instituted once the diagnosis of GCA is established.

Glucocorticoids have inhibitory effects on a broad range of specific immune responses. Their effectiveness in GCA is well established by years of use.  Daily dosing is more effective than alternate day dosing. This response usually occurs within two to four weeks after the institution of therapy. The diagnosis should be reevaluated in patients who are resistant to adequate steroid therapy. Steroid withdrawal can begin once clinical remission has been induced. Relapses are seen more frequently in the first year or two of the disease.

Adverse effects of corticosteroids are glucose intolerance or frank diabetes mellitus (condition of abnormal glucose levels in blood) and infections. Patients should be aware and watchful so as to report symptoms suggestive of diabetes or infection. Use of glucocorticoids may also predispose to, or worsen preexisting, osteoporosis (abnormal bone density condition) especially in postmenopausal women and older men. Use of calcium supplements, vitamin D, hormone replacement therapy and/or bisphosphonates are can be helpful in preventing bone mineral loss.

Relapses often necessitate increased dosage or prolonged steroid treatment. Some researchers have suggested that the addition of methotrexate may be steroid-sparing while others have not demonstrated any benefit. However the routine addition of methotrexate to glucocorticoid therapy for GCA is not recommended. The efficacy of other cytotoxic drugs, dapsone, antimalarials, etanercept, and penicillamine has not been studied adequately although they have been reported to be helpful in some case reports.

The finding of an increased risk of visual loss in patients with GCA and thrombocytosis (increase of the number of platelets in the blood), has led some to suggest the addition of drugs  like aspirin for patients with high platelet counts, but there is not a lot of data to prove that this may reduce brain/skull problems.

Henoch-Schonlein Purpura (HSP)

Henoch-Schönlein purpura (HSP) is a systemic vasculitis that causes the blood vessels in the skin to become inflamed, causing red spots. When the blood vessels in the skin get inflamed, they can bleed, causing a rash that is called purpura. This rash is typically seen on the lower legs or arms. The specific skin lesion is characterized by the tissue deposition of an immune system product, called IgA immunoglobulin, which is also found in kidneys of patients with a renal disease, called IgA nephropathy.

HSP occurs more often in children than in adults, and many cases follow an upper respiratory tract infection (infection in your sinuses and /or lungs). Half of affected children are under age five, although kidney involvement is more likely to be severe in older children. Compared to children, adults had more severe and frequent kidney involvement.

Symptoms  occur over a period of days to several weeks: skin rash, joint aches and pains, usually in knees and ankles, occasional swelling, abdominal pain and renal disease manifesting mostly as hematuria (blood in your urine), proteinuria (abnormal excretion of proteins in urine), edema (selling) or alteration in the volume of urine. The hematuria may be noticed as red or tea-colored or cola-colored urine or the amount may be so small that it can only be seen under a microscope. The brain or the lung may also be involved in HSP.

Gastrointestinal symptoms are present in the majority of patients including abdominal pain that is frequently associated with vomiting. The pain typically develops within eight days of the appearance of the rash. Bleeding of the gastrointestinal duct presenting with black or bright red color in stools is seen in these patients. Although rare, more serious complications may develop like intussusception, a situation in which one portion of the bowel slides into the next creating an obstruction in the bowel, leading to swelling, inflammation, and decreased blood flow to the intestines involved or inflammation of other organs leading to pancreatitis, cholecystitis, and entero nephrotic pathy.

Renal (kidney) involvement is common, occurring in 30-70 % of patients. Kidney disease is usually noted after the onset of systemic symptoms. More  marked findings may also occur including nephrotic syndrome, a situation characterized by abnormal excretion of proteins and lipids in urine, swelling (edema), low level of albumin in blood and hyperlipidemia.   High blood pressure (hypertension) and acute kidney failure may also be seen. Worsening of the kidney symptoms and biopsy-confirmed worsening of the kidney lesions may be observed in patients with repeated attacks of rash or hematuria (blood in the urine).

Even though the symptoms of HSP make it easier to diagnose in children, confirmation of the diagnosis of HSP requires evidence of tissue deposition in the skin or kidney of IgA immunoglobulin. Renal biopsy is another method to establish the diagnosis, but is reserved for patients in whom the diagnosis is uncertain or in whom there is evidence of more severe renal involvement.

The overall outcome is good in most patients. All of the manifestations of active HSP usually resolve spontaneously, although recurrent episodes of skin rash and hematuria may be seen. Among those with

kidney involvement, only a minority have persistent disease. The kidney prognosis is excellent in most patients. However some patients will have persistent protein in their urine, high blood pressure, and renal insufficiency. It is estimated that HSP accounts for approximately 3% of cases of end-stage kidney disease in children. Poor renal prognosis is more common among those with the nephrotic syndrome, renal insufficiency, and more advanced findings on biopsy.

Recurrences are common, occurring in approximately one-third of patients.  Since complete recovery occurs in 94% of children and 89 % of adults, respectively, most patients receive no specific therapy. There is suggestive evidence that corticosteroids enhance the rate of resolution of the arthritis and abdominal pain, although they do not appear to prevent recurrent disease.

However, specific treatment is recommended in patients with marked proteinuria (protein in the urine) and/or impaired kidney function during the acute episode. A kidney biopsy can be performed to reveal the severity of the lesions which appears to be the best indicator of prognosis. Advanced disease, usually defined as crescentic nephritis, is treated with a regimen consisting of pulse intravenous methylprednisolone followed by oral prednisone.

Other regimens that have been evaluated in children with kidney disease include corticosteroids and azathioprine and multidrug regimens such as corticosteroids, cyclophosphamide, and dipyridamole, or corticosteroids, cyclophosphamide, heparin/warfarin, and dipyridamole. However, since spontaneous recovery is often observed in these patients, it remains unknown whether these regimens are superior to no or less aggressive therapy.

Plasmapheresis has also been used in a number of patients with severe disease although its efficacy is uncertain. Intravenous immune globulin has been tried in a small number of patients with heavy proteinuria and a progressive decline in kidney function.

Kidney transplantation can be performed in those patients who progress to end-stage kidney disease, although recurrent disease can occur. This appears to be more likely in patients with aggressive initial disease who progressed to end-stage kidney disease in less than three years after the onset of HSP. Therefore it is recommended the transplantation to be delayed for 12-24 months after the disappearance of the rash. Some observations suggest that the risk of recurrent disease also may be higher in living-related donors.

Hypersensitivity Vasculitis

Hypersensitivity vasculitis is often used to describe different types of vasculitis related to drug reactions, skin disorders or allergic vasculitis; however this is not always the correct use of the term. 

Given the wide range of symptoms, the varying definitions and frequent incorrect use of the term, the American College of Rheumatology made a list of criteria for the classification of hypersensitivity vasculitis. Three or more of these criteria are needed to determine that a patient with some form of vasculitis is defined as specifically having hypersensitivity vasculitis.  The criteria are:

  (1) older than 16 years of age

  (2) use of a drug before the development of symptoms

  (3) skin rash

  (4) biopsy of the skin rash that shows neutrophils, a type of white blood cells, around a small vessel

It should be noted that having three of these criteria does not always distinguish hypersensitivity vasculitis from other forms of vasculitis, particularly when the only or first symptom of vasculitis is a skin rash.

The presence of skin vasculitis, usually red spots, is the main symptom in hypersensitivity vasculitis. A biopsy of these skin spots reveals inflammation of the small blood vessels, called a leukocytoclastic vasculitis.

Hypersensitivity vasculitis may be caused by a specific drug or occur in association with an infection, but it may also be idiopathic, meaning there is no known cause.  Although drugs are the most common cause, drug-induced vasculitis is a poorly defined disorder.

There are no symptoms or tests that prove hypersensitivity vasculitis results directly from a particular drug.  The drugs that are most frequently listed as being associated with the development of hypersensitivity vasculitis include: penicillin, cephalosporin, sulfonamide, some medicines used to control blood pressure (loop and thiazide-type diuretics), phenytoin and allopurinol.  Infections that may be associated with hypersensitivity vasculitis include hepatitis B or C virus, chronic infection with bacteria and HIV virus.

Symptoms: The major symptoms of hypersensitivity vasculitis, in addition to a skin rash, are joint pains and increasing size of lymph nodes.  Lymph nodes are located in several places, but particularly along the neck, and supply special cells to the bloodstream that help remove bacteria from the body.  In most patients, symptoms begin 7 to 10 days after the exposure to the drug or

infection, but can be as short as two to seven days in some people. 

Organ involvement in addition to the skin rash is very rare, but can be severe.  Kidney inflammation and even more rarely liver, lung, heart and brain injury have occurred in patients with hypersensitivity vasculitis. The kidney inflammation is usually mild.

Symptoms of kidney involvement may not be noticed by the patient, but can be evaluated by a doctor by looking at a urine sample for small amounts of blood and protein.  Kidney failure is not common, but can occur particularly with heavy or prolonged exposure to the suspected drug or infection.  Kidney failure can be ‘acute', meaning there is a fast loss of kidney function, but supportive treatment with dialysis (mechanical cleansing of the blood) can be done for a few days or weeks and kidney function returns.  In some cases, ‘chronic' kidney failure occurs, meaning that there is an ongoing need for dialysis because the kidneys do not recover their normal function. 

Treatment: If a drug may have caused the hypersensitivity vasculitis, then discontinuation of that specific drug usually leads to the disappearance of symptoms within a few days or weeks.  If an infection may have caused the hypersensitivity, then treatment of the infection usually results in the disappearance of symptoms. 

In some patients, especially those with ongoing infections such as hepatitis B or C, there may be ongoing or ‘chronic' symptoms of hypersensitivity vasculitis.  Drugs used to manage the skin rash and joint pains associated with hypersensitivity vasculitis might include corticosteroids and/or nonsteroidal anti-inflammatory drugs. 

In patients with more severe or ongoing skin rashes that are not due to infection, drugs such as colchicine, antihistamines, and dapsone (or a combination of these drugs) may be helpful to control symptoms.  Patients with disease in organs beyond the skin should be referred to a specialty doctor such as a nephrologist if the kidneys are involved. 

6.06

Kawasaki Disease

Kawasaki Disease is a rare vasculitis, which strikes children. Over 4,000 children develop it each year. 80% of patients are under the age of 5. Patients usually begin with a fever that lasts at least five days. 

Symptoms may include red eyes, lips, and mouth; rash; swollen and red hands and feet; and swollen lymph nodes.  The disorder affects the mucus membranes, lymph nodes, walls of the blood vessels, and the heart.  The most important aspect of the disease is the heart's involvement. The disease can cause inflammation of blood vessels in the coronary arteries, which can lead to aneurysms.  Kawasaki is the leading cause of acquired heart disease in children.

Cause: There is no known cause of the disease.

Symptoms:  Kawasaki disease often begins with a high and persistent fever greater than 102°F, often as high as 104°F. A persistent fever lasting at least five days is considered a hallmark sign. The fever may persist steadily for up to two weeks and is not very responsive to normal doses of acetaminophen or ibuprofen.

Diagnosis is usually based on evaluation of classic symptoms. Possible diagnostic tests include a complete blood count (CBC), ESR, Electrocardiogram, Echocardiogram, Chest x-ray and urinalysis.

Immediate treatment is critical to avoid permanent damage to the coronary arteries and heart. Standard treatment includes high doses of Intravenous gamma globulin.  The patient's condition usually greatly improves within 24 hours of treatment.

With early recognition and treatment, full recovery can be expected. However, 2% of patients die from complications of coronary blood vessel inflammation. Patients who have had Kawasaki disease should have an echocardiogram every 1-2 years to screen for heart problems.

6.06

Microscopic Polyangiitis (MPA)

Microscopic polyangiitis (MPA) is an inflammation of the medium and small vessel walls that can affect different parts of the body including (but not limited to) the kidney, lungs, sinuses.   Patients with MPA usually have the disease in their kidney and it is essentially indistinguishable from the kidney disease that patients with classic Wegener's granulomatosis (WG) often have. 

The principal difference between MPA and WG is the absence of a specific type of inflammation called granulomatous inflammation that is what distinguishes WG from other forms of vasculitis, although the clinical features are similar in these two diseases. Patients presenting with kidney disease in the absence of disease in other parts of the body are generally classified as "renal-limited" vasculitis.

In the United States, the typical MPA patient is a middle-aged white male or female, but in fact the disease may occur in people of all ages, both genders, and all ethnic backgrounds.

The presenting symptoms include fever, joint and muscle pains, weakness, lack of energy and weight loss. Hematuria, the abnormal presence of blood in the urine, swelling and a decrease in the amount of urine may be manifestations of renal involvement which is one of the most frequently involved organs in MPA. In fact approximately 90% of patients with MPH have involvement of their kidneys (called glomerulonephritis, as called because the inflammation affects the part of the kidney that filters fluid to make urine and is called the glomerulous). Lung symptoms like shortness of breath, coughing up blood, and/or chest pain are experienced in about half of the patients. MPA is the most common cause of the so called pulmonary-renal syndrome, which is the common combination of inflammation and disease symptoms in both the lung and the kidney. 

In MPA the symptoms of the upper airway (sinuses, ears) can occur, but are less common or milder than kidney or lung symptoms. The eyes and the nervous system can also affected by the disease.  A skin rash, usually with purplish bumps and spots mostly in the lower extremities, is sometimes seen. The stomach and intestinal tract can also be affected by the disease, resulting in pain or an alteration of the color of stools into black or bright red as a result of bleeding. 

Over 80% of patients with MPA have ANCA.

The diagnosis of MPA is established with the biopsy, a small piece of tissue that is taken from the affected organ(s).  The biopsy tissue is looked at under microscopes and reveals vasculitis. A skin or kidney biopsy is typically preferred, but can also be done in other organs such as the intestine. 

The treatment of MPA is essentially the same as in WG especially when the major organs injured by the disease are the kidney and/or lung. 

Induction therapy is the initial step for all patients diagnosed with MPA in order to reduce the inflammation of the disease.  A minimum of three to six months induction phase is needed for most patients to reduce or get rid

of the inflammation, which is often known as ‘getting the disease into remission."

Maintenance therapy is the following step and is meant to keep the disease in remission as long as possible and therefore reduce the chance that the inflammation will return (called a disease relapse).  Maintenance therapy can be continued for 12 to 18 months, sometimes longer.  For patients who never completely get their disease into remission or who experience several disease relapses, ongoing maintenance therapy may be continued indefinitely.

Immunosuppressive regimens used in the induction phase of treatment usually include a combination of corticosteroids (prednisone) with either daily oral cyclophosphamide (Cytoxan) or monthly intravenous (given into a vein at a doctor's office or hospital) cyclophosphamide (Cytoxan). 

Low-dose weekly oral methotrexate has been tried in patients with MPA without severe inflammation from the disease. Corticosteroids monotherapy is not generally considered for remission induction, since the reported remission rate is much lower. Patients who are dialysis-dependent at presentation seem to benefit from plasmapheresis. Although no controlled studies have been performed, patients with pulmonary hemorrhage may be treated with plasmapheresis also.

Maintenance therapy includes immunosuppressive agents such as mycophenolate mofetil, Rituximab, azathioprine, methotrexate, Cyclosporine, etanercept.  Trimethoprim-sulfamethoxazole is an antibiotic agent used also relating with the fact that disease activity has been associated with both infection and the chronic nasal carriage of Staphylococcus aureus.

Treatment-associated toxicity: Cyclophosphamide treatment is associated with important toxicity.  Women may stop having their menstrual cycles (periods) and men may have lowered or no sperm produced; either of which may not return to normal even after stopping treatment. 

Inflammation of the bladder (cystitis), bladder cancer, myelodysplasia, (disorder of the function of the bone marrow, the part of the bone related to the production of blood cells) and lymphoma (type of cancer of the lymph nodes) can also occur with use of this drug. Some studies suggest that monthly intravenous cyclophosphamide (given by vein) may be as effective as daily oral treatment with the same drug in controlling symptoms and reducing inflammation, while reducing the overall cumulative dose and chance of side effects.  

Long-term use of corticosteroids use can include cataracts (a clouding of the natural lens, the part of the eye responsible for focusing light and producing clear, sharp images), diabetes mellitus (disorder of the glucose levels in blood), osteoporosis (thin bones), fractures (small breaks in bones), aseptic necrosis of bone (condition in which poor blood supply to an area of bone leads to bone death) and severe pain and inflammation of the stomach.

Pneumonia and other infections are serious complications of any mmunosuppressive therapy, including cyclophosphamide and corticosteroids. 

Prophylaxis: Given the toxicities of cyclophosphamide, prophylactic therapy is usually provided with specific drugs to help prevent specific types of pneumonia, permanent loss off periods (menstrual cycles) in women of child-bearing, and bladder cancer.

Given the toxicities of prolonged steroid use, prophylactic treatments may also be provided to help prevent for mouth infections, stomach pain and inflammation and bone loss. 

The major lifetime effects in patients with MPA result from the combined effects of irreversible organ damage (such as kidney failure or lung scarring from the disease as well as the consequences of the cumulative dose and total period of each immunosuppressive therapy, whether utilized for initial disease, maintenance of remission, or management of relapses of the disease. Therefore the natural history of the disease is diverse among patients.

6.06

Polyarteritis Nodosa (PAN)

Polyarteritis Nodosa (PAN) is a vasculitis disease, which affects the small and medium-sized arteries.   PAN commonly affects the skin, heart, kidneys and central nervous system.

Cause:  There is no known cause of PAN.

Symptoms include fever, fatigue, weakness, loss of appetite, and weight loss. Muscle and joint aches are common. The skin may show rashes, swelling, ulcers, and lumps.

Other symptoms include abdominal pain and gastrointestinal bleeding (occasionally is mistaken for inflammatory bowel disease).  Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness. Central nervous system involvement may cause strokes or seizures. Kidney involvement can produce varying degrees of renal failure.  Involvement of the arteries of the heart may cause a heart attack, heart failure, and inflammation of the sack around the heart (pericarditis).

There is no specific test to diagnosis PAN. Diagnosis is based upon physical examination, lab tests and biopsy of affected area.  Most patients with PAN have elevated ESRs.  Proteinuria (protein in the urine) is common among patients with kidney involvement.

The American College of Rheumatology 1990 criteria for the classification of Polyarteritis Nodosa

1. Weight loss of > 4 kg since beginning of illness
2. Livedo reticularis
3. Testicular pain or tenderness
4. Myalgias, weakness, or leg tenderness
5. Mononeuropathy or polyneuropathy
6. Development of hypertension
7. Elevated BUN or creatinine unrelated to dehydration or obstruction
8. Presence of hepatitis B surface antigen or antibody in serum
9. Arteriogram demonstrating aneurysms or occlusions of the visceral arteries
10. Biopsy of small or medium-sized artery containing granulocytes

Treatment will vary based on patient symptoms, disease activity, organ involvement and lab test results.

Treatment of PAN has improved dramatically in the past couple of decades. Before the availability of effective therapy, untreated PAN was usually fatal within weeks to months. Most deaths occurred as a result of kidney failure, heart or gastrointestinal complications. However, effective treatment is now

available for PAN. After diagnosis, patients are treated with high doses of corticosteroids. Other immunosuppressive drugs are also added for patients who are especially ill. In most cases of PAN now, if diagnosed early enough the disease can be controlled, and often cured.

The newly proposed regimen for patients with PAN associated with hepatitis B, consists of 2 weeks of prednisone to control the vasculitis, followed by plasmapheresis to remove immune complexes, and accompanied by antiviral therapy with lamivudine to rid the patient of the hepatitis B infection. The long-term value of anti-viral therapy for polyarteritis nodosa associated with hepatitis C is not established.

Polymyalgia Rheumatica (PMR)

Polymyalgia rheumatica (PMR) is a condition that is frequently linked to giant cell arteritis (GCA) (link). PMR occurs in about 50 % of patients who have GCA, while approximately 15% of patients with PMR develop GCA. There may be a common genetic component between the two disorders.  PMR is almost exclusively a disease that affects older adults and is rarely diagnosed in people under the age of 50 years. 

Symptoms: Symptoms of PMR almost always include aching and morning stiffness in the shoulders, hips, neck and mid-body. These symptoms usually affect both sides of the body the same, but can be stronger on one side than the other.  Having difficulty with pain, stiffness and movement of the shoulders and hips can result in trouble with things such as getting dressed. Some patients also complain of general tiredness, weakness, weight loss (without trying to lose weight), and a low fever (a high spiking fever is rare).

Inflammation in the bones and joints cause the discomfort and stiffness (difficulty in moving) found among patients with PMR. Some patients develop swelling or fluid retention (edema) of the hands, wrists, ankles, and top of the feet. The edema usually occurs with other signs of PMR but can be the only symptom experienced.

Decreased ability to fully move the shoulders, neck and hips is frequent. Muscle strength is usually normal and the tenderness found about the shoulders is more likely due to inflammation in the shoulder bones. However, muscle weakness may become a problem over time because of the lack of use due to pain and stiffness. 

The characteristic laboratory finding in both PMR and GCA is an elevation in the erythrocyte sedimentation rate. This rate measures how fast a patient's red blood cells settle when placed in a small tube. 

Routine x-rays (radiographs) of joints with the disease rarely reveal any abnormalities, while magnetic resonance imaging (MRI) examinations can confirm the presence of inflammation. Ultrasounds and Positron emission tomography (PET scanning) have also been used to confirm the PMR inflammation.

Since there is no specific test for PMR, a checklist that requires a certain group of symptoms and laboratory characteristics is used by doctors to make the diagnosis.

There is considerable overlap between PMR and GCA but patients with "pure" PMR lack the symptoms of GCA. Thus, a biopsy of the temporal artery, which is diagnostic for GCA, is not necessary in patients with PMR unless there are symptoms suggestive of GCA.

Treatment: The beneficial effect of corticosteroids (predinsone) in patients with PMR has been established by a combination of clinical experience and several research studies. Initial treatment most often starts with a dose of prednisone between 7.5 and 20 mg/day. Patients usually respond quickly but the dose is increased if the symptoms are not well controlled within one week. In some patients a single daily dose of prednisone does not provide relief from evening or night-time pain or stiffness while a divided dose (2 times a day, usually 12 hours apart) may be more helpful in reducing symptoms. The effective steroid dose is maintained for 2-4 weeks after the symptoms have resolved. The dose is then gradually lowered and stopped, with careful monitoring for return of symptoms.

Return of symptoms (relapse) occurs in as many as 25-50% of patients. Relapse is more likely to occur if the steroid dose is decreased too fast.  If symptoms return, restarting or increasing the dose of corticosteroids is appropriate.

Side effects with corticosteroids: The risk of diabetes (abnormal glucose blood levels) and risk of fractures (small cracks) in the bones of the back, hip and neck are increased, especially with frequent use of this therapy.  In patients who require corticosteroid treatment for more than six months, an assessment of bone density is suggested to test for osteoporosis (loss of bone thickness). To help protect bones from fractures and osteoporosis, calcium and vitamin D are often taken regularly, and sometimes drugs such as bisphosphonates are given.

In patients who have side-effects from or a long history of taking corticosteroids, the use of methotrexate may allow the corticosteroid dose to be eliminated or lowered, however this has only been suggested by some, but not all, studies. Anti-inflammatory drugs such as ibuprofen can also be used to decrease painful symptoms, especially when symptoms are only mild, and may also help avoid use of corticosteroid treatment

Effort must be focused at control of symptoms with a minimum of drug-induced side effects.  In most patients, symptoms of PMR will eventually end (over a period of months to years) and corticosteroid therapy can be discontinued.

Rheumatoid Vasculitis (RV)

Rheumatoid vasculitis (RV) refers to patients with rheumatoid arthritis, a chronic disease with painful inflammation of the joints, who also develop inflammatory disease in small and medium-sized blood vessels.  RV most commonly occurs in the skin as venulitis or capillaritis, meaning the very smallest blood vessels are affected by inflammation from the disease.  RV occurs in approximately 2 to 5 % of patients who have rheumatoid arthritis.

The reason why RV develops in some patients with rheumatoid arthritis and not others is not clear. Genetic factors may be involved.  Viral infections and drug reactions have been suggested as causes of RV, but there is little research to support this.  Some research suggests that long time use of drugs such as corticosteroids, gold compounds, penicillamine and azathioprine that are used to treat rheumatoid arthritis can cause the development of RV.   However, this may not be true and difficult to determine because more use of these drugs is probably because of more severe or long standing rheumatoid arthritis, both of which may also be associated with the development of RV. 

RV typically occurs in patients who have had rheumatoid arthritis for a long time. In one study, for example, the average time between the diagnosis of rheumatoid arthritis and the onset of RV symptoms was 13.6 years. Patients with rheumatoid arthritis seem more likely to develop RV when they have high rheumatoid factor levels (a specific laboratory finding for rheumatoid arthritis) and disease of at least one year's duration. Males with rheumatoid arthritis are more likely (2 to 4 times more likely) than females with rheumatoid arthritis to develop RV.

The manifestations of RV can involve many of the body's organs, including the skin, nerves to the hands and feet, blood vessels of the fingers and toes, and the eyes. Skin vasculitis is the most common manifestation of RV, occurring in as many as 90% of patients. Inflammation of the small blood vessels in the skin results in the development of red spots on the skin.  When the eyes are involved, there is usually inflammation of the white part of the eye (scleritis). 

The heart can also be affected by the disease, which can cause inflammation of the external part of the heart (pericarditis) and abnormal heart rate (arrhythmia).  These symptoms put these patients at a higher risk for having a heart attack (myocardial infarction).

Patients with rheumatoid arthritis should see a physician if they develop new or worsened symptoms such as weight loss, fever, and lack of energy, any new symptoms beyond the usual joint symptoms.  A blood test for specific antibodies that are directed against the inner layer of blood vessels (endothelial cells) are present in approximately 75% of patients with RV compared to only 15to 20%

of those with rheumatoid arthritis alone.  Therefore, this blood test may be checked regularly in

patients with any of these new or worsened symptoms.

Diagnosis: Many of the drugs used to treat RV have a number of side effects; therefore it is important to be sure of the diagnosis before treatment is started (see treatment section below).  The diagnosis of RV almost always requires a biopsy of tissue affected by the disease; an inflamed nerve or a kidney if there are clinical signs of kidney involvement, for example. 

In rare cases RV may affect large blood vessels.  If this happens or your doctor thinks it may have happened, then ‘pictures' will be taken so that the vessels can be evaluated.  Some of these pictures require that you drink something called ‘contrast' material.  This material shows up on the picture and helps to show different parts of the inside of your body. This test is called contrast angiography and is especially useful to help determine the location and appearance of large vessels that may be affected by the disease.  

Biopsy-proven RV, even if only in one organ, requires aggressive therapy. The limited data specifically related to RV suggest that most such patients should be treated with the same or similar drugs that are used in other primary systemic vasculitides such as combination therapy with cytotoxic drugs (usually cyclophosphamide) and corticosteroids. Cyclophosphamide given through the veins (intravenous) once a month has been used with success, although daily oral therapy with the same drug may also be effective. 

A variety of different types of corticosteroid treatment have been used. Patients with aggressive RV disease are usually begun on pulse methylprednisolone (corticosteroids given through the veins once a day for several days) followed by daily oral prednisone. Other drugs have been explored in patients with RV. Some patients have done well with azathioprine and corticosteroids. However azathioprine may be better used to maintain a remission after initial cyclophosphamide therapy helps to control the disease and its symptoms. Methotrexate and tumor necrosis factor (TNF), also known as infliximab, have also been used.  However, some patients have developed RV while on these drugs for the treatment of rheumatoid arthritis.  Since RV most often occurs when there is very active rheumatoid arthritis, aggressive treatment usually helps to control symptoms of both rheumatoid arthritis and vasculitis. 

Supportive care is also very important. Smoking has been associated with an increased

risk for rheumatoid arthritis and for RV. Therefore smoking cessation is essential in any rheumatoid arthritis patient, especially those with RV.   Good skin care may also prevent infectious complications of skin rashes in RV.

Limited data are available concerning the outcome of patients with RV, although they usually have worse and more ongoing symptoms than those with rheumatoid arthritis who do not have RV.

Takayasu's Arteritis (TA)

Takayasu's arteritis (TA) is a vasculitis that is found more commonly in women than in men, with 80% to 90% of people with the disease being women. People who get the disease are usually between the ages of 10 and 40 years when the disease starts. The disease affects people around the world, but is most common in Asians, especially in Japan.

TA primarily affects the aorta, the major vessel that carries blood that supplies oxygen to the body. Inflammation from the disease may be localized to one small part of the aorta, or may involve the entire length of the vessel. As the disease progresses, other major blood vessels may also become affected with inflammation. The abdominal aorta, the part of the aorta that runs through the middle of the body and supplies blood to the stomach area, and lung arteries are involved in about one half (50%) of patients.  The inflammatory process causes the walls of the aorta to become thick, which makes it difficult for blood to flow easily.  This can cause many different symptoms because it depends on where in the body the blood flow is affected and which organs may not get all the blood that they need to work right. 

The cause of or reason that TA develops is not understood, although genetics have been associated with increased frequency in several studies.

Symptoms: Symptoms that are common when TA first develops include lack of energy, weight loss, weakness and low-grade fever.  Symptoms that affect the blood vessels take time to develop. As the disease progresses the arms and legs may become cool, and pain may develop with use. Other symptoms include joint pain, muscle weakness and pain, and skin lesions.  Symptoms of the disease can also include chest pain, shortness of breath and coughing up blood if the lungs become affected. Shortness of breath can develop if the heart is affected.  Anemia (low blood count) is present in most patients and results in a general feeling of weakness and tiredness.

Involvement of the major blood vessel that carries blood to the head and brain (the carotid artery) causes decreased blood flow that can lead to dizziness, fainting, headaches, seizures and difficulties thinking and remembering. Difficulty with seeing may also develop.  Stomach pain, diarrhea, and stomach bleeding can result from inflammation in the stomach or intestines. Inflammation of the vessels that supply the heart (coronary arteries) can cause chest pain and puts patients at higher risk of having a heart attack (myocardial infarction).  

Diagnosis:  In order for doctors to see what is happening to the aorta and other blood vessels, several different types ‘pictures' may be taken.  Radiography (x-ray) of the chest may be used to look for changes in the aorta.  Other types of pictures require that you drink or be given a shot of

something called ‘contrast' material.  This material shows up on the picture and helps to show different parts of the inside of your body. These tests are called contrast angiography or arteriography, and they are especially useful to help determine the location and appearance of vessels affected by the disease.  

Computed tomographic (CT) or magnetic resonance (MR) imaging scans of the chest, abdomen, head and neck, or other areas may also be used, particularly so that doctors can evaluate large arteries. Positron emission tomography (PET scanning) can also be used to take pictures of the aorta and other large vessels.  Several of these types of tests and pictures may be needed in order to diagnose and manage TA. 

The main drug used for treatment of TA is corticosteroids, also known as glucocorticoids.   Treatment with this medicine usually stops this disease from getting worse by reducing the inflammation associated with the disease.  However, use of this medicine, especially for long periods of time, can cause many adverse reactions or side-effects.

Approximately one-half of all patients with TA have disease that is ongoing and does not get completely better with the use of corticosteroids.  When this is the case, other drugs such as methotrexate, cyclophosphamide, and azathioprine may reduce inflammation and be helpful in controlling symptoms of the disease.  A new drug that may be helpful for patients with TA who do not get better with the treatments listed above is called an anti-tumor necrosis factor alpha (anti-TNF) agent. However, there are not many studies of this type of drug in this disease, although a large study is planned.

Several types of medical procedures may be used to help repair damaged blood vessels and to restore blood flow if major damage from the disease occurs.  Angioplasty is a medical procedure in which a balloon is used to open narrowed or blocked blood vessels.  A by-pass graft may also be done, which is a surgery performed to redirect a clogged or severely damaged vessel. 

Prognosis: TA is a chronic disease. The degree of new disease involvement over time can be quite different between patients, and the inflammation may never completely go away.  In some patients the disease eventually ‘burns out' and symptoms and inflammation do not ever return. 

Wegener's Granulomatosis (WG)

Wegener's Granulomatosis (WG) is an uncommon disease that affects about 1 in 20,000 to 1 in 30,000 people.  Symptoms are due to inflammation that can affect many tissues in the body, including blood vessels (vasculitis).  It is also considered a disease of abnormal immune function.

There is no known cause of WG; but it is not contagious, and there is no evidence it is hereditary.  It is systemic, meaning it affects the body as a whole.  It affects the upper (sinuses and nose), and lower (lungs), respiratory system and frequently involves the kidneys, lungs, eyes, ears, throat, skin and other body organs.  For reasons not clear, blood vessels in those areas may become inflamed and clusters of certain cells (granulomas) may occur. 

WG is an uncommon disease, which can occur at any age.  It most often occurs in the 4th and 5th decade of life.  Patients are divided equally between males and females.  It appears that Caucasians are far more commonly affected than other racial groups.

Onset of WG may be indolent, slow moving with few symptoms, or have a rapid and severe onset.  About 90% of patients have symptoms of a 'cold' ' 'runny nose' or sinusitis that fail to respond to the usual therapeutic measures and last considerably longer than the usual upper respiratory tract infection.  Other symptoms include nasal membrane ulcerations and crusting, saddle-nose deformity, inflammation of the ear with hearing problems, inflammation of the eye with sight problems, cough (with or without the presence of blood), pleuritis, (inflammation of the lining of the lung), rash and/or skin sores, fever, lack of energy, weakness, fatigue, loss of appetite, weight loss, arthritic joint pain, night sweats, and blood in urine which may or may not be indicated by a change in urine color.

Be aware that not all WG patients experience all symptoms.  Different patients experience different symptoms, and the severity of the disease is also different for each WG patient.  If any of the above symptoms persist, consider a possible diagnosis of WG and arrange to have a complete evaluation, including health history, physical exam, laboratory studies, including a urinalysis and an ANCA test.

Diagnosis is established by clinical and laboratory findings such as the ANCA blood test, other blood and urine tests, x-rays, and tissue biopsy, if needed.

The treatment of WG can be divided into two stages: firstly, the induction of disease remission, and secondly, the maintenance of disease remission.  Medication usually consists of cytotoxic agents (a form of chemotherapy), using relatively low doses of Cyclophosphamide (Cytoxan),

and/or Methotrexate and/or Azathioprine (Imuran), and glucocorticoids (Prednisone).

Treatment will vary based on patient symptoms, disease activity, organ involvement and lab test results. Patients with kidney involvement and more severe WG are commonly prescribed Cyclophosphamide and Prednisone as initial treatment.  Ideally, the use of Cyclophosphamide will be limited to a three to six month period and then replaced, based on kidney function, by Methotrexate or Azathioprine.

Those with milder forms of WG are commonly prescribed Methotrexate and Prednisone.  These medications will be reduced over time, and even eliminated, if the patient remains in a stable remission.  WG patients may also be prescribed calcium supplements and other medications to prevent osteoporosis from extended prednisone use.  Many patients will also be prescribed the antibiotic Bactrim to help prevent secondary lung infections with a dangerous "bug" called Pneumocystis carinii pneumonia (PCP).  In addition, there is some evidence that Bactrim, used cautiously, can have the beneficial effects of reducing relapses and upper airway infections.

Effective treatment should include a "team approach" with medical specialists according to the patient's organ involvement.  It is common for a WG patient to regularly see the following Doctors: Nephrologist (Kidney), Otolaryngologist (Ear, Nose/Sinus, Throat), Ophthalmologist (Eye), Pulmonologist (Lung), and always a Rheumatologist or Immunologist.  Other specialists are involved as needed.

There is no cure for Wegener's Granulomatosis, but early diagnosis and proper treatment will be effective and the disease can be brought into remission with complete absence of all signs of disease.

Long-term remission can be induced and maintained with medications, close management and regular lab tests to help monitor the disease.  Treatment can produce symptom-free intervals of 5 to 20 years or more.  Some patients will achieve a drug-free remission.  However, relapses are common but can be caught at their earliest and most treatable stage, for most patients, by paying attention to patient symptoms and lab tests.  WG patients in remission must not hesitate to see a doctor if any WG symptoms return or if they are not feeling well.

Research into new medications, treatment options and the cause of WG are being investigated at leading medical centers throughout the world. 

French Brochures - Vasculitis Foundation Soutine - Information - Recherche

Qu’est-ce qu’une vascularite ?

Une vascularite est définie par l’existence d’une inflammation des vaisseaux sanguins, c’est-à-dire des artères, veines et/ou capillaires. Les parois des vaisseaux deviennent alors anormalement épaisses ou, à l’inverse s’amincissent et se dilatent. Le flux sanguin est alors perturbé et se ralentit, et des caillots peuvent se former, qui finissent par boucher les vaisseaux atteints.

Les organes ou tissus irrigués par ces vaisseaux malades ne sont donc plus suffisamment oxygénés et peuvent souffrir. On parle de souffrance ischémique, qui peut aller jusqu’à une nécrose de l’organe ou du tissu atteint.

Le groupe des vascularites comporte en fait plusieurs maladies différentes, qui sont toutes rares. Elles sont classées en fonction de la taille et de la localisation des vaisseaux atteints. Elles peuvent toucher des individus de tous les âges. Certaines sont bénignes et guérissent spontanément, mais la plupart d’entre elles nécessitent des traitements spécifiques. Elles mettent parfois en jeu le pronostic vital. La durée des traitements varie d’une maladie à l’autre, mais peut parfois être très prolongée, pendant plusieurs années, voire à vie.

Leurs causes sont pour la plupart totalement inconnues, mais avec les progrès effectués en médecine ces 20 dernières années, il existe en tout cas aujourd’hui des traitements qui ont permis d’en améliorer de façon très significative l’évolution.

La poursuite des recherches biomédicales et thérapeutiques dans le domaine des vascularites reste essentielle pour en améliorer la prise en charge et finalement en guérir.

A quel âge peut-on voir apparaître une vascularite ?

Les vascularites peuvent survenir à tous les âges de la vie, aussi bien chez l’enfant que chez la personne âgée. Certaines tranches d’âge sont cependant davantage touchées en fonction du type de vascularite concernée.

Quels sont les symptômes des vascularites ?

Les manifestations cliniques sont très variables d’un patient à l’autre et d’une vascularite à l’autre. Les signes les plus fréquents qui doivent faire penser à ces maladies, lorsqu’ils sont associés, sont :

- la fatigue

- une sinusite chronique traînante

- une toux et/ou un essoufflement au moindre effort

- une bronchite ou une pneumonie traînante

- un asthme difficile à contrôler ou d’apparition tardive dans la vie

- des douleurs articulaires

- des douleurs abdominales et/ou des saignements digestifs

- des problèmes rénaux (présence de sang ou d’albumine dans les urines)

- des problèmes neurologiques (maladresse, perte de la force musculaire dans les mains et/ou les pieds, engourdissements)

- difficultés ou troubles visuels

- maux de tête inhabituels

- éruptions cutanées

- fièvre

Quelle est la cause de ces vascularites ?

Dans la majorité des cas, on ne connaît pas la cause de ces maladies. Il s’agit pour la plupart d’entre elles de maladies auto-immunes, c’est-à-dire liéed à un emballement du système immunitaire naturel qui, en plus de défendre normalement l’organisme contre d’éventuels agents infectieux, s’attaque aussi et par erreur aux cellules du patient lui-même. Ce dérèglement du système immunitaire est probablement provoqué par la combinaison de plusieurs facteurs, comme l’exposition à des agents de l’environnement (certaines poussières par exemple) ou à certaines infections (comme le virus de l’hépatite B ou C), et d’un terrain génétique prédisposant, qui à lui seul n’est pas suffisant pour déclencher la maladie.

Comment fait-on le diagnostic de ces vascularites ?

Le diagnostic de vascularite peut être fortement suspecté par le médecin devant la combinaison de plusieurs signes cliniques et des résultats d’examens complémentaires, tests sanguins et urinaires, bilan radiologique, comportant des radiographies des poumons et des sinus, un scanner ou parfois une angiographie. D’autres tests plus spécifiques, comme la recherche de certains auto-anticorps peuvent aussi aider à faire le diagnostic. La biopsie d’un tissu ou d’un organe atteint, lorsque cela est possible, peut permettre un diagnostic plus précis.

Peut-on guérir d’une vascularite ?

Un diagnostic précoce suivi d’un traitement adapté, prescrit par des médecins qui connaissent bien ces maladies, permettent dans la majorité des cas d’obtenir ce que l’on appelle une rémission, c’est-à-dire une disparition de tous les signes cliniques d’activité de la maladie. On ne parle pas encore de guérison à ce stade car un certain nombre de patients peut faire des rechutes et ce pendant encore plusieurs années. Selon la vascularite concernée, des traitements prolongés sont donc parfois nécessaires pour limiter ce risque de rechute.

Quel est le traitement des vascularites ?

Le traitement varie en fonction du type de vascularite concernée et de la sévérité des atteintes constatées. Il est également adapté à l’état général du patient, pour en limiter les effets secondaires potentiels. Les traitements comportent souvent des corticoïdes et/ou des médicaments immunosuppresseurs, mais parfois aussi des antibiotiques, des échanges plasmatiques ou des perfusions d’agents dits ‘biologiques’, par exemple des anticorps dirigés spécifiquement contre certains globules blancs (anticorps monoclonaux) ou des immunoglobulines intraveineuses.

Les principes fondateurs de l’association

Aider la recherche médicale et améliorer l’information dans le domaine des vascularites  systémiques, afin de permettre un diagnostic précoce de ces maladies et la mise en œuvre rapide d’un traitement approprié et efficace.

Association fondée en Mai 1986.

A propos de la Vasculitis Foundation

La Vasculitis Foundation (VF, auparavant Wegener’s Granulomatosis Association) a été fondée en 1986 par Marilyn Sampson, une patiente atteinte de granulomatose de Wegener, infirmière de métier.

La VF s’était donnée comme mission première de lever l’isolement et la sensation de solitude ressentie par les patients atteints de ces maladies rares et graves, et de leurs familles. La VF aide ainsi les patients et leur proches à lutter plus efficacement contre ces maladies, en les aidant à rester positifs et optimistes dans leur avenir, à travers notamment le partage de leur vécu de la maladie avec de nombreux patients qui sont guéris ou en voie de guérison.

Soutien / Information / Recherche

Un diagnostic précoce et la mise en œuvre rapide d’un traitement approprié et efficace peuvent améliorer le pronostic de ces maladies. Les objectifs de la VF, à travers son site internet, ses publications périodiques bimensuelles, ses brochures d’information, ses contacts étroits avec de nombreux chercheurs et médecins spécialistes, ses réunions, ainsi que par la création et le soutien de nombreux groupes de patients à travers le monde, sont d’améliorer la connaissance, d’aider à promouvoir la recherche, et de pouvoir fournir toutes les informations nécessaires, dans le domaine des vascularites, aux patients, à leur famille et à leurs amis pour leur permettre de mieux combattre la maladie.

La VF soutient également la recherche médicale, grâce aux cotisations de ses membres et à leurs dons, par l’attribution de bourses et prix annuels pour aider à financer des programmes de recherche épidémiologique, d’identification des causes et des mécanismes des vascularites, et de développement de nouvelles thérapeutiques stratégies thérapeutiques. A ce jour, plus de 12 projets de recherche ont ainsi pu être soutenus financièrement.

Experts médicaux

De nombreux médecins spécialistes et experts dans le domaine des vascularites ont accepté, à travers le monde entier, de rédiger des articles pour la VF, d’aider et de soutenir l’association. Ils ont notamment accepté que leur adresse soit mise sur le site et dans les publications de la VF en tant qu’experts pouvant donner gratuitement à tout médecin le souhaitant des conseils, des recommandations, ou un simple avis pour la prise en charge de tout patient atteint de vascularite.

www.VasculitisFoundation.org

Les Vascularites Systémiques

Angéite cérébrale primitive

Artérite de Takayasu

Artérite giganto-cellulaire (maladie de Horton)

Granulomatose de Wegener

Maladie de Behçet

Maladie de Buerger (thromboangéite oblitérante)

Maladie de Kawasaki

Périartérite noueuse

Polyangéite microscopique

Pseudo-polyarthrite rhizomélique

Purpura rhumatoïde

Syndrome de Churg et Strauss

Vascularite cryoglobulinémique

Vascularite d’hypersensibilité

Vascularite de la polyarthrite rhumatoïde

PO Box 28660

Kansas City, MO 64188

800.277.9474

816.436.8211 Phone/Fax

www.VasculitisFoundation.org

A propos de l’association Wegener Infos Vascularites et du Groupe Français d’Etude des Vascularites

Le Groupe Français d’Etude des Vascularites est un groupe de recherche thérapeutique et biomédicale créé au début des années 80 en France. Ce groupe participe au conseil scientifique de l’association Française des patients atteints de vascularite, Wegener Infos et Vascularites, fondée en Janvier 2006 et qui est le correspondant en France de la VF.

GFEV - Groupe Français d'Etude des Vascularites,

Centre national de référence Maladies systémiques et auto-immunes rares, en particulier les vascularites nécrosantes et la sclérodermie systémique,

Pôle de Médecine du Pr Guillevin,

Hôpital Cochin,

27, rue du faubourg Saint-Jacques

75679 Paris cedex 14,

France

Tél. : 33 1 58 41 14 61 or 33 1 58 41 13 21

Fax : 01 58 41 14 60

E-mail : groupe.vascularite@cch.aphp.fr

Site web : http://www.vascularites.org

WEGENER INFOS ET VASCULARITES

Mr Bernard PIASTRA

Route de Vichy

03120 Isserpent

Tél. : 33 4 70 41 35 58

E-mail : wegener.infos.vascularites@orange.fr

Arterite De Takayasu

Qu’est-ce que l’artérite de Takayasu ?

L’artérite de Takayasu est une maladie très rare qui fait partie du groupe des vascularites systémiques et qui affecte les vaisseaux sanguins de gros et moyen calibres. Les vaisseaux atteints sont principalement l’aorte (la principale artère partant du cœur) et ses premières branches de division, notamment les artères distribuant le sang vers le cerveau, les bras, l’abdomen et les membres inférieurs.

Quelle est la cause de l’artérite de Takayasu ?

On ne connaît pas la cause exacte de cette maladie, et il n’y en a probablement pas qu’une seule. Il existe peut-être une susceptibilité génétique individuelle, mais ce n’est pas une maladie héréditaire et elle n’est pas contagieuse.

Qui sont les personnes atteintes d’artérite de Takayasu ?

L’artérite de Takayasu touche surtout les femmes (80–90% des sujets atteints). Elle est rencontrée dans tous les continents du globe, mais elle est plus fréquente en Asie, et en particulier au Japon. Les premières atteintes liées à la maladie se constituent en général entre 10 et 40 ans.

Quels sont les symptômes de la maladie ?

Les premières manifestations de la maladie peuvent être de la fièvre, une fatigue excessive, un amaigrissement involontaire, une faiblesse générale. L’atteinte des artères peut entraîner une froideur, une cyanose et/ou des douleurs des mains et pieds, liées à la mauvaise circulation du sang artériel.

Il existe parfois des douleurs articulaires, une faiblesse musculaire, des lésions cutanées, des douleurs thoraciques, un essoufflement à l’effort, une différence des chiffres de tension artérielle d’un bras par rapport à l’autre, des troubles de l’équilibre, des maux de tête, des crises d’épilepsie et/ou des troubles de la concentration ou de la mémoire. Des troubles de la vue peuvent aussi survenir. Une anémie (baisse du taux d’hémoglobine) est notée chez la plupart des patients. L’inflammation des vaisseaux de l’abdomen peut entraîner des douleurs abdominales, des diarrhées voire des hémorragies digestives. L’inflammation des artères coronaires peut causer de l’angine de poitrine, c’est-à-dire des douleurs thoraciques à l’effort, et parfois même être à l’origine d’un infarctus du myocarde.

Comment fait-on le diagnostic ?

La radiographie de thorax montre parfois des anomalies de la taille de l’aorte. Les vaisseaux sanguins sont cependant beaucoup mieux analysés à l’aide d’une angiographie (ou artériographie), d’une angio-IRM, d’un angio-scanner et/ou d’un TEP-scanner, qui préciseront mieux quels sont les vaisseaux atteints.

Quel est le pronostic de la maladie ?

L’artérite de Takayasu est une maladie chronique. L’inflammation persiste souvent indéfiniment et impose donc un traitement prolongé, souvent à vie. Chez quelques patients, la maladie finit toutefois par «s’éteindre» totalement après quelques mois ou années de traitement. Les symptômes et l’inflammation disparaissent alors.

Quel est le traitement de l’artérite de Takayasu?

Le traitement de base de l’artérite de Takayasu repose sur les corticoïdes, pour stopper l’évolution de la maladie et réduire l’inflammation des vaisseaux. Cependant, ce traitement seul n’est suffisant que chez la moitié des patient(e)s. Un autre traitement (un immunosuppresseur) doit dans ces cas être prescrit, en association aux corticoïdes, afin de réduire l’inflammation et de contrôler les symptômes.

Qu’est-ce qu’une vascularite ?

Une vascularite est définie par l’existence d’une inflammation des vaisseaux sanguins, artères, veines et/ou capillaires. Le groupe des vascularites comporte plusieurs maladies différentes, qui mettent parfois le pronostic vital en jeu. Leurs causes sont pour la plupart totalement inconnues, mais avec les progrès effectués en médecine ces 20 dernières années, des traitements existent aujourd’hui qui ont permis d’en améliorer de façon très significative l’évolution.

La poursuite des recherches biomédicales et thérapeutiques dans le domaine des vascularites reste essentielle pour en améliorer la prise en charge et finalement en guérir.

A propos de la Vasculitis Foundation

La Vasculitis Foundation (VF, auparavant Wegener’s Granulomatosis Association) est la plus importante association internationale de soutien et d’information des patients atteints de vascularite et de leur entourage.

Les objectifs de la VF, à travers son site internet, ses publications périodiques, ses brochures d’information, ses contacts étroits avec de nombreux chercheurs et médecins spécialistes, ses réunions, ainsi que par la création et le soutien de nombreux groupes de patients à travers le monde, sont d’améliorer la connaissance, d’aider à promouvoir la recherche, et de pouvoir fournir toutes les informations nécessaires, dans le domaine des vascularites, aux patients, à leur famille et à leurs amis pour leur permettre de mieux combattre la maladie.

A propos de l’association Wegener Infos Vascularites et du Groupe Français d’Etude des Vascularites

Le Groupe Français d’Etude des Vascularites est un groupe de recherche thérapeutique et biomédicale créé au début des années 80 en France. Ce groupe participe au conseil scientifique de l’association Française des patients atteints de vascularite, Wegener Infos et Vascularites, créée en Janvier 2006 et qui est le correspondant en France de la VF.

Arterite Giganto-Cellulaire

Qu’est-ce que l’artérite giganto-cellulaire?
L’artérite giganto-cellulaire (ou maladie de Horton) fait partie du groupe des vascularites systémiques. Elle affecte les vaisseaux sanguins de gros et moyen calibres, en particulier les vaisseaux de la tête, dont les artères temporales.
 

Quelle est la cause de l’artérite giganto-cellulaire?
On ne connaît pas la cause exacte de cette maladie. On sait par contre qu’elle n’est pas contagieuse.
 

Qui sont les personnes atteintes d’artérite giganto-cellulaire?
Les sujetsde race blanche (on dit parfois ‘caucasiens’) âgés de plus de 50 ans sont les plus touchés par cette maladie, dont la fréquence augmente très clairement avec l’âge (âge moyen de survenue = 72 ans). Les femmes sont 2 à 3 fois plus concernées que les hommes, ceci s’expliquant en partie par la simple différence d’espérance de vie entre les deux sexes observée dans la population générale.
 

Quels sont les symptômes de la maladie?
Les symptômes de la maladie s’installenthabituellement de façon progressive en quelques semaines. Il peut s’agir de fièvre, de fatigue, de faiblesse générale et/ou d’un amaigrissement involontaire.

La moitié des patients ont des douleurs dans les mâchoires lors de la mastication (appelée claudication des mâchoires).  Des troubles de la vue peuvent aussi survenir, parfois de façon soudaine et révélant la maladie. Ils sont liés à une atteinte inflammatoire uni- ou bilatérale des vaisseaux de la rétine ou des nerfs optiques.
 

La pseudo-polyarthrite rhizomélique (PPR), caractérisée par des douleurs des épaules et du bassin, est une maladie très proche, souvent associée d’ailleurs, puisque 40–50% des patients
atteintsd’artérite giganto-cellulaire ont des symptômes de PPR.
 

Comment fait-on le diagnostic?
Le diagnostic d’artérite giganto-cellulaire repose sur la biopsie d’artère temporale, qu’il faut effectuer dès que le diagnostic est suspecté. La plupart des patients ont un syndrome inflammatoire biologique marqué, avec une élévation de la CRP et de la vitesse de sédimentation (VS). Certains examens radiologiques peuvent mettre en évidence l’inflammation des gros vaisseaux atteints, comme l’échographie des artères temporales, le TEP-scanner, ou plus rarement un simple scanner de l’aorte (aortite) ou une angio-IRM. L’examen du fond d’œil est important pour dépister des troubles de la vascularisation (ischémie) de la rétine ou du nerf optique qui imposent un traitement en urgence.
 

Quel est le pronostic de la maladie?

Les corticoïdes sont habituellement très efficaces. Des rechutes peuvent cependant survenir, le plus souvent dans la première ou deuxième année suivant le diagnostic.
 

Quel est le traitement de l’artérite giganto-cellulaire ?
Les corticoïdes doivent être prescrits et débutés dès que le diagnostic est établi ou fortement suspecté en cas d’atteinte ophtalmologique. La biopsie de l’artère temporale peut être reportée et réalisée quelques jours plus tard sans aucun problème, lorsque le traitement doit être débuté en urgence pour préserver la vue. La prise de corticoïdes doit être quotidienne et durer plusieurs mois, à des doses lentement dégressives, même si la disparition complète des symptômes est en général obtenue dès la 2 ème–4ème semaine de traitement.
 

Qu’est-ce qu’une vascularite?
Une vascularite est définie par l’existence d’une inflammation des vaisseaux sanguins, artères, veines et/ou capillaires. Le groupe des vascularites comporte plusieurs maladies différentes, qui mettent parfois le pronostic vital en jeu. Leurs causes sont pour la plupart totalement inconnues, mais avec les progrèseffectués en médecine ces 20 dernières années, des traitements existent aujourd’hui qui ont permis d’en améliorer de façon très significative l’évolution.
 

La poursuite des recherches biomédicales et thérapeutiques dans le domaine des vascularites reste essentielle pour en améliorer la prise en charge et finalement en guérir.
 

A propos de la Vasculitis Foundation
La Vasculitis Foundation (VF, auparavant Wegener’s Granulomatosis Association) est la plus importante association internationale de soutien et d’information des patients atteints de vascularite et de leur entourage.
 

Les objectifs de la VF, à travers son site internet, ses publications périodiques, ses brochures d’information, ses contacts étroits avec de nombreux chercheurs et médecins spécialistes, ses réunions, ainsi que par la création et le soutien de nombreux groupes de patients à travers le monde, sont d’améliorer la connaissance, d’aider à promouvoir la recherche, et de pouvoir fournir toutes les informations nécessaires, dans le domaine des vascularites, aux patients, à leur famille et à leurs amis pour leur permettre de mieux combattre la maladie.
 

A propos de l’association Wegener Infos Vascularites et du Groupe Français d’Etude des Vascularites
Le Groupe Français d’Etude des Vascularitesest un groupe de recherche thérapeutique et biomédicale créé au début des années 80 en France. Ce groupe participe au conseil scientifique de l’association Française des patients atteints de vascularite, Wegener Infos et Vascularites, créée en Janvier 2006 et qui est le correspondant en France de la VF.

French Translation: Angeite Primitive Du Systeme Nerveux Central (SNC)

Qu’est-ce que l’angéite primitivedu SNC ?

L’angéite, ou vascularite, primitive du système nerveux central, est une maladie extrêmement rare, liée à une inflammation des vaisseaux sanguins du cerveau et/ou de la moëlle épinière et de leurs enveloppes (les leptoméninges).

Quelle est la cause del’angéite primitivedu SNC?

On ne connaît pas la cause exacte de cette maladie. On sait par contre qu’elle n’est pas contagieuse.
 

Certains médecins et chercheurs pensent que des facteurs d’environnement, notamment des infections par des virus, «banals» ou à l’inverse inconnus et non identifiés, pourraient stimuler de façon excessiveet inadaptée le système naturel de défense immunitaire, qui s’attaqueraitalors par erreur aux vaisseaux sanguins de petit et moyen calibres du cerveau.

Qui sont les personnes atteintes d’angéite primitivedu SNC?

L’angéite primitivedu SNCdoit être distinguée de l’angiopathie cérébrale par vasoconstriction réversible, qui est beaucoup plus fréquente et bénigne et que l’on considérait il y a quelques années comme une forme bénigne d’angéite primitivedu SNC. En fait, c’est une maladie est très différente car elle est liée à des spasmes plus ou moins durables des artères cérébrales, mais sans inflammation. 

L’angéite primitivedu SNC peut toucher aussi bien l’homme que la femme, tandis que l’angiopathie cérébrale par vasoconstriction réversible touche surtout les femmes jeunes,  en particulier celles qui sont hypertendues et/ou sujettes à de fréquents maux de tête. L’angéite primitivedu SNC concerne principalement l’adulte âgé de 40 à 60 ans, mais de très jeunes enfants ou les personnes âgées peuvent aussi être atteints.
 

Quels sont les symptômes de la maladie?

Les symptômes les plus fréquemment constatés au cours des angéites primitivesdu SNC sont la confusion mentale, les maux de tête, des changements du caractère et de la personnalité, une faiblesse musculaire et/ou une paralysied’un ou plusieurs membres comme on peut l’observer chez les personnes faisant une «attaque cérébrale», c’est-à-dire un accident vasculaire cérébral (hémiplégie, paraplégie…).

D’autres manifestations sont parfois observées comme des crises d’épilepsie, une hémorragie méningée ou cérébrale, un coma ou une baisse brutale de la vision. Les symptômes s’installent en général de façon progressive sur plusieurs semaines ou mois, mais peuvent parfois apparaître
beaucoup plus rapidement, voire du jour au lendemain. Aucun de ces signes cliniques n’est spécifique de l’angéite primitivedu SNC, car de très nombreuses autres maladies ou circonstances peuvent se présenter exactement de la même manière.

Comment fait-on le diagnostic?
La plupart des patients devront avoir une artériographie cérébrale et/ou une biopsie du cerveau. Ces examens sont dits «invasifs», mais lorsqu’ils sont effectués par des médecins expérimentés, ils comportent assez peu de risques. Une IRM et un scanner du cerveau sont également réalisés de façon habituelle. Cependant, aucun de ces examens ne permet de confirmer le diagnostic à 100%. Il faut surtout éliminer toutes les autres maladies du SNC qui peuvent causer des symptômes similaires à ceux de l’angéite primitive du SNC. Ces maladies sont nombreuses et bien plus fréquentes que l’angéite primitivedu SNC, mais elles ne sont pas pour autant plus
faciles à diagnostiquer.
 

Quel est le pronostic de la maladie?

L’angéite primitivedu SNC est difficile à traiter.Des immunosuppresseurs et/ou des corticoïdes sont souvent prescrits, avec parfois de bons résultats.

Comme son nom l’indique, l’angiopathie cérébrale par vasoconstriction réversible régresse habituellement en quelques semaines, avec un traitement assez léger, voire spontanément. Il peut toutefois persister des séquelles, notamment si la période de spasme artériel a été prolongée ou sévère.
 

Quel est le traitement de l’angéite primitivedu SNC?

Certains patients répondent très bien à des traitements comportant de fortes doses de corticoïdes. D’autres ont besoin de recevoir des immunosuppresseurs en association à la corticothérapie.

Des inhibiteurs calciques (vasodilatateurs) sont prescrits chez les patients atteints d’angiopathie cérébrale par vasoconstriction réversible, parfois en association avec une courte corticothérapie initiale.
 

Qu’est-ce qu’une vascularite?

Une vascularite est définie par l’existence d’une inflammation des vaisseaux sanguins, artères, veines et/ou capillaires. Le groupe des vascularites comporte plusieurs maladies différentes, qui mettent parfois le pronostic vital en jeu. Leurs causes sont pour la plupart totalement inconnues, mais avec les progrèseffectués en médecine ces 20 dernières années, des traitements existent aujourd’hui qui ont permis d’en améliorer de façon très significative l’évolution.
 

La poursuite des recherches biomédicales et thérapeutiques dans le domaine des vascularites reste essentielle pour en améliorer la prise en charge et finalement en guérir.

A propos de la Vasculitis Foundation
 

La Vasculitis Foundation (VF, auparavant Wegener’s Granulomatosis Association) est la plus importante association internationale de soutien et d’information des patients atteints de vascularite et de leur entourage.

Les objectifs de la VF, à travers son site internet, ses publications périodiques, ses brochures d’information, ses contacts étroits avec de nombreux chercheurs et médecins spécialistes, ses réunions, ainsi que par la création et le soutien de nombreux groupes de patients à travers le monde, sont d’améliorer la connaissance, d’aider à promouvoir la recherche, et de pouvoir fournir toutes les informations nécessaires, dans le domaine des vascularites, aux patients, à leur famille et à leurs amis pour leur permettre de mieux combattre la maladie.

A propos de l’association Wegener Infos Vascularites et du Groupe Français d’Etude des Vascularites

Le Groupe Français d’Etude des Vascularitesest un groupe de recherche thérapeutique et biomédicale créé au début des années 80 en France. Ce groupe participe au conseil scientifique de l’association Française des patients atteints de vascularite, Wegener Infos et Vascularites, créée en Janvier 2006 et qui est le correspondant en France de la VF.

French Translation: Purpura Rhumatoide

Qu’est-ce que le purpura rhumatoïde ?

Le purpura rhumatoïde ou purpura de Henoch-Schönlein est une vascularite systémique caractérisée par une inflammation des vaisseaux sanguins, en particulier de la peau où l’on peut constater des petits points rouges (purpura) lors des poussées. Elle évolue par poussées successives, en général durant 2 à 3 semaines seulement avant de complètement disparaître, mais parfois elle devient chronique avec des poussées itératives pendant plusieurs mois ou années.

Quelle est la cause du purpura rhumatoïde ?

On ne connaît pas la cause exacte de cette maladie. On sait par contre qu’elle n’est pas contagieuse, même si on retrouve souvent une histoire d’angine, de sinusite ou de bronchite dans les jours ou semaines précédant les poussées.

Qui sont les personnes atteintes de purpura rhumatoïde ?

Le purpura rhumatoïde affecte plus souvent les enfants, surtout ceux âgés de moins de 5 ans, que les adultes. La maladie varie un peu en fonction de l’âge, notamment les atteintes rénales qui sont plus fréquentes et sévères chez les grands enfants et les adultes.

Quels sont les symptômes de la maladie ?

Les symptômes peuvent survenir par vagues successives et répétés durant plusieurs jours à semaines. Ils peuvent comporter une atteinte de la peau (éruption de purpura), douleurs et gonflements articulaires, surtout des genoux et chevilles. L’atteinte digestive est très fréquente, avec des douleurs abdominales, des nausées et parfois des saignements. Une atteinte rénale est possible, chez 30–70% des patients (excès de protéines et/ou présence de sang dans les urines, voire insuffisance rénale).

Comment fait-on le diagnostic ?

Le diagnostic de purpura rhumatoïde est relativement simple à faire cliniquement chez l’enfant. Une biopsie de peau et/ou du rein, en cas d’atteinte rénale, peut aider à confirmer le diagnostic.  

Quel est le pronostic de la maladie ?

L’évolution spontanée est en général rapidement favorable chez la lupart des patients. Des récidives peuvent cependant survenir, rapprochées et transitoires, mais parfois pendant plusieurs mois ou année.

Le pronostic de l’atteinte rénale est également bon la plupart du temps, mais certains patients peuvent garder longtemps des traces de protéines dans leurs urines ou rester hypertendus, ou insuffisants rénaux. Chez l’enfant, le purpura rhumatoïde représente 3% de l’ensemble des causes d’insuffisance rénale chronique.

Quel est le traitement du purpura rhumatoïde?

La plupart des manifestations disparaissent spontanément. Lorsqu’ils persistent un peu trop et/ou en cas d’atteinte rénale ou digestive sévère, un traitement par corticoïdes, et parfois par immunosuppresseur, est nécessaire. En cas d’atteinte rénale très sévère, des échanges plasmatiques peuvent aussi être effectués en complément, mais dont l’efficacité n’est pas certaine malgré tout.

Qu’est-ce qu’une vascularite ?

Une vascularite est définie par l’existence d’une inflammation des vaisseaux sanguins, artères, veines et/ou capillaires. Le groupe des vascularites comporte plusieurs maladies différentes, qui mettent parfois le pronostic vital en jeu. Leurs causes sont pour la plupart totalement inconnues, mais avec les progrès effectués en médecine ces 20 dernières années, des traitements existent aujourd’hui qui ont permis d’en améliorer de façon très significative l’évolution.

La poursuite des recherches biomédicales et thérapeutiques dans le domaine reste essentielle pour en améliorer la prise en charge et finalement en guérir.

A propos de la Vasculitis Foundation

La Vasculitis Foundation (VF, auparavant Wegener’s Granulomatosis Association) est la plus importante association internationale de soutien et d’information des patients atteints de vascularite et de leur entourage.

Les objectifs de la VF, à travers son site internet, ses publications périodiques, ses brochures d’information, ses contacts étroits avec de nombreux chercheurs et médecins spécialistes, ses réunions, ainsi que par la création et le soutien de nombreux groupes de patients à travers le monde, sont d’améliorer la connaissance, d’aider à promouvoir la recherche, et de pouvoir fournir toutes les informations nécessaires, dans le domaine des vascularites, aux patients, à leur famille et à leurs amis pour leur permettre de mieux combattre la maladie.

A propos de l’association Wegener Infos Vascularites et du Groupe Français d’Etude des Vascularites

 Le Groupe Français d’Etude des Vascularites est un groupe de recherche thérapeutique et biomédicale créé au début des années 80 en France. Ce groupe participe au conseil scientifique de l’association Française des patients atteints de vascularite, Wegener Infos Vascularites, créée en Janvier 2006 et qui est le correspondant en France de la VF.

French Translation: Vascularite D'Hypersensibilite

Qu’est-ce qu’une vascularite d’hypersensibilité ?

Une vascularite d’hypersensibilité est liée à une inflammation des vaisseaux sanguins de petit calibre, en particulier de la peau. Elle est déclenchée la plupart du temps par la prise d’un médicament ou par une réaction allergique à un produit ou un facteur irritant, qui est parfois une infection virale ‘banale’. Fréquemment, on ne sait pas quel est l’agent responsable.

Quelle est la cause des vascularites d’hypersensibilité ?

Dans la plupart des cas, c’est la prise d’un médicament qui est à l’origine de cette affection, qui apparait en général au bout de quelques jours à semaines après avoir commencé à le prendre, mais parfois après plusieurs mois ou années. Dans certains cas, il peut s’agir d’une réaction transitoire liée à une infection virale banale et bénigne. Ce n’est cependant pas une maladie contagieuse, ni héréditaire.

Qui sont les personnes atteintes de vascularite d’hypersensibilité ?

La définition des vascularites d’hypersensibilité est très discutée, de même que son nom, puisqu’on l’appelle parfois aussi vascularite allergique ou vascularite leucocytoclasique liée à la prise d’un médicament… Elle peut survenir à tout âge. Trois des 4 critères de classification suivants sont nécessaires pour pouvoir retenir le diagnostic de cette affection : âge supérieur à 16 ans ; prise récente d’un médicament avant l’apparition des premiers signes ; éruptions cutanées ; présence d’un infiltrat inflammatoire des petits vaisseaux à la biopsie des lésions cutanées.

Quels sont les symptômes de la maladie ?

Le principal symptôme de la maladie est une éruption cutanée, faites de petits points rouges, indolores, exceptionnellement sources de démangeaisons. Cette éruption peut toucher tout le corps ou seulement une partie, notamment les jambes, et survient le plus fréquemment dans les 7 à 10 jours suivant l’introduction d’un nouveau médicament.

D’autres manifestations sont possibles, comme une fièvre modérée, des douleurs articulaires et/ou l’augmentation transitoire de la taille des ganglions lymphatiques du cou ou sous les bras.

Exceptionnellement, d’autres organes peuvent être touchés, en particulier les reins (urines foncées, présence de sang), les poumons (asthme, pneumonie diffuse) ou le cœur (péricardite).

Comment fait-on le diagnostic ?

Le diagnostic de vascularite d’hypersensibilité repose sur des arguments cliniques et la notion d’apparition des lésions après l’introduction d’un nouveau médicament. La biopsie des lésions cutanées permet de confirmer l’existence d’une vascularite.

Quel est le pronostic de la maladie ?

L’évolution spontanée est en général rapidement favorable après l’arrêt définitif du médicament en cause. Des récidives peuvent parfois survenir, en particulier si la cause n’a pas pu être identifiée.

Quel est le traitement des vascularites d’hypersensibilité?

La plupart des symptômes disparaissent spontanément. Lorsqu’ils persistent un peu trop longtemps et/ou en cas d’atteinte rénale, un traitement par corticoïdes peut être nécessaire. Dans certains cas, d’autres traitements sont associés, comme la dapsone, la colchicine ou des antihistaminiques.

Qu’est-ce qu’une vascularite ?

Une vascularite est définie par l’existence d’une inflammation des vaisseaux sanguins, artères, veines et/ou capillaires. Le groupe des vascularites comporte plusieurs maladies différentes, qui mettent parfois le pronostic vital en jeu. Leurs causes sont pour la plupart totalement inconnues, mais avec les progrès effectués en médecine ces 20 dernières années, des traitements existent aujourd’hui qui ont permis d’en améliorer de façon très significative l’évolution.

La poursuite des recherches biomédicales et thérapeutiques dans le domaine des vascularites reste essentielle pour en améliorer la prise en charge et finalement en guérir.

A propos de la Vasculitis Foundation

La Vasculitis Foundation (VF, auparavant Wegener’s Granulomatosis Association) est la plus importante association internationale de soutien et d’information des patients atteints de vascularite et de leur entourage.

Les objectifs de la VF, à travers son site internet, ses publications périodiques, ses brochures d’information, ses contacts étroits avec de nombreux chercheurs et médecins spécialistes, ses réunions, ainsi que par la création et le soutien de nombreux groupes de patients à travers le monde, sont d’améliorer la connaissance, d’aider à promouvoir la recherche, et de pouvoir fournir toutes les informations nécessaires, dans le domaine des vascularites, aux patients, à leur famille et à leurs amis pour leur permettre de mieux combattre la maladie.

A propos de l’association Wegener Infos Vascularites et du Groupe Français d’Etude des Vascularites

Le Groupe Français d’Etude des Vascularites est un groupe de recherche thérapeutique et biomédicale créé au début des années 80 en France. Ce groupe participe au conseil scientifique de l’association Française des patients atteints de vascularite, Wegener Infos et Vascularites, créée en Janvier 2006 et qui est le correspondant en France de la VF.

French Translation: Vascularites Cryoglobulinemiques

Qu’est-ce qu’une cryoglobulinémie ?

Une cryoglobulinémie est définie la présence anormale dans le sang d’une ou plusieurs protéine(s) (cryoglobuline) qui peut/peuvent précipiter au froid, ralentissant alors la circulation sanguine dans les petits vaisseaux, et pouvant donc provoquer une ischémie puis une nécrose des tissus mal irrigués. Cette protéine anormale est fabriquée par des lymphocytes B (il s’agit en fait d’anticorps très particuliers réagissant à des températures inférieures à 37°C) chez des patients atteints de diverses maladies auto-immunes ; de certaines maladies du sang, comme le myélome multiple ou certaines leucémies ; en association à une infection par le virus de l’hépatite C ; ou encore au cours de certaines infections pulmonaires. Dans ce dernier cas, elle est en général fabriquée de façon très transitoire.

Quelle est la cause des cryoglobulinémies ?

La cryoglobulinémie de type I est habituellement associée aux maladies du sang, comme le myélome multiple. Les cryoglobulinémies mixtes, de type II et III, sont elle associées dans 70–90% à une infection par le virus de l’hépatite C. En l’absence de cause identifiée, on parle de cryoglobulinémie ‘essentielle’.

Qui sont les personnes atteintes de cryroglobulinémie mixte ?

L’infection par le virus de l’hépatite C concerne avant tout les toxicomanes par voie intraveineuse qui partagent leurs seringues. L’infection peut cependant aussi se transmettre par contamination sanguine (transfusions de produits sanguins autrefois, piqûre accidentelle avec du matériel contaminé) et plus rarement par voie sexuelle. Toutefois, dans un quart des cas, on ne sait pas quel a été le mode de contamination.

Quels sont les symptômes des vascularites cryoglobulinémiques ?

Tous les individus ayant une cryoglobuline ne développent pas de vascularite. Les cryoglobulinémies, de type II et III surtout, se compliquent en effet seulement dans 5–20% des cas de vascularite des petits vaisseaux, notamment de la peau, des reins ou des nerfs.

Les symptômes sont représentés par des éruptions cutanées sur les membres, en particulier les jambes (purpura), une insuffisance rénale liée à une glomérulonéphrite et/ou une neuropathie périphérique, responsable de douleurs et/ou de troubles de la sensibilité dans les pieds et/ou les mains.

Comment fait-on le diagnostic ?

La détection dans le sang et l’identification précise du type de cryoglobuline fabriquée est un élément essentiel du diagnostic. La sérologie de l’hépatite C est importante également.

Quel est le traitement des vascularites cryoglobulinémiques ?

Le traitement dépend du type de cryogobulinémie et donc de sa cause, ainsi que de la sévérité de la maladie. Des corticoïdes et parfois des immunosuppresseurs sont souvent nécessaires.

Lorsque la quantité de cryoglobuline fabriquée anormalement est très abondante, le sang peut devenir très épais et visqueux, ralentissant alors considérablement la circulation de l’oxygène dans l’organisme (on parle de syndrome d’hyperviscosité). Dans ces cas, des échanges plasmatiques doivent être réalisés, combinés à un traitement immunosuppresseur.

En cas d’infection par le virus de l’hépatite C, un traitement antiviral est préconisé en association aux corticoïdes, ou plus rarement comme seul traitement. Il existe plusieurs médicaments aujourd’hui pour traiter cette infection, en particulier l’interféron alpha, mais qui malheureusement n’est pas efficaces chez tous les patients. De plus, des rechutes de la vascularite ou de l’hépatite chronique surviennent parfois à l’arrêt de ces traitements ou un peu après leur arrêt.

Qu’est-ce qu’une vascularite ?

Une vascularite est définie par l’existence d’une inflammation des vaisseaux sanguins, artères, veines et/ou capillaires. Le groupe des vascularites comporte plusieurs maladies différentes, qui mettent parfois le pronostic vital en jeu. Leurs causes sont pour la plupart totalement inconnues, mais avec les progrès effectués en médecine ces 20 dernières années, des traitements existent aujourd’hui qui ont permis d’en améliorer de façon très significative l’évolution.

La poursuite des recherches biomédicales et thérapeutiques dans le domaine des vascularites reste essentielle pour en améliorer la prise en charge et finalement en guérir.

A propos de la Vasculitis Foundation

La Vasculitis Foundation (VF, auparavant Wegener’s Granulomatosis Association) est la plus importante association internationale de soutien et d’information des patients atteints de vascularite et de leur entourage.

Les objectifs de la VF, à travers son site internet, ses publications périodiques, ses brochures d’information, ses contacts étroits avec de nombreux chercheurs et médecins spécialistes, ses réunions, ainsi que par la création et le soutien de nombreux groupes de patients à travers le monde, sont d’améliorer la connaissance, d’aider à promouvoir la recherche, et de pouvoir fournir toutes les informations nécessaires, dans le domaine des vascularites, aux patients, à leur famille et à leurs amis pour leur permettre de mieux combattre la maladie.

A propos de l’association Wegener Infos Vascularites et du Groupe Français d’Etude des Vascularites

Le Groupe Français d’Etude des Vascularites est un groupe de recherche thérapeutique et biomédicale créé au début des années 80 en France. Ce groupe participe au conseil scientifique de l’association Française des patients atteints de vascularite, Wegener Infos et Vascularites, créée en Janvier 2006 et qui est le correspondant en France de la VF.

Granulomatose De Wegener

Qu’est-ce que la granulomatose de Wegener?
La granulomatose de Wegener est une maladie rare qui fait partie du groupe des vascularites systémiques. Cette maladie affecte environ une personne sur 20.000 à 30.000 dans le monde. Les symptômes sont liés à l’inflammation des vaisseaux sanguins, qui peut toucher différents organes dans le corps.On considère parfois la granulomatose de Wegener comme une maladie auto-immune, c’est-à-dire liée à un emballement du système de défense immunitaire qui s’attaquerait alors aux tissus et aux organes du patient lui-même.
 

Quelle est la cause de la granulomatose de Wegener?
On ne connaît pas la cause exacte de cette maladie, et il n’y en a probablement pas qu’une seule. On sait par contre qu’elle n’est pas contagieuse.
 

Qui sont les personnes atteintes de la granulomatose de Wegener?
La granulomatose de Wegener peut survenir à tout âge. Cependant, elle apparaît surtout chez des adultes âgés de 40 à 60 ans, aussi bien des femmes que des hommes. Elle atteint presque exclusivement les personnesà peau blanche (que l’on dit parfois ‘Caucasiens’).
 

Quels sont les symptômes de la maladie?
La granulomatose de Wegener est une maladie systémique, c’est-à-dire qu’elle peut affecter tous les organes et tissus du corps. Elle touche cependant avec prédilection les voies respiratoires hautes (nez, sinus, oreilles) et basses (poumons, bronches) et fréquemment les reins, la peau, les yeux et les nerfs.
 

Environ 90% des patients signalent avoir un «rhume» traînant, le «nez qui coule» ou une sinusite qui ne guérit pas malgré plusieurs traitements antibiotiques succesifs. Les autres signes peuvent être des ulcérations de la muqueuse nasale, avec parfois d’importantes croûtes nasales, des déformations du nez (en nez de boxeur), une inflammation des oreilles ou des yeux, de la toux, des douleurs thoraciques, une éruption cutanée, de la fièvre, de la fatigue, une sensation de faiblesse générale et persistante, un manque d’appétit, un amaigrissement, des douleurs articulaires, ou du sang dans les urines.

Tous les patients n’ont cependant pas l’ensemble de ces symptômes.  
 

Comment fait-on le diagnostic?
Le diagnostic repose sur la combinaison d’arguments cliniques et biologiques comme la détection d’auto-anticorps (les ANCA) dans le sang des patients. D’autres tests sanguins et urinaires, des radiographies ou des scanners, et des biopsies de tissus ou d’organe sont habituellement nécessaires, pour le diagnostic et choisir le traitement le plus adapté.
 

Quel est le pronostic de la maladie?

Il n’y a pas de traitement efficace à 100%, mais une prise en charge thérapeutique précoce et adaptée dans des centres spécialisés permet dans la majorité des cas d’obtenir une rémission de la maladie. On parle de rémission, plutôt que de guérison, car les rechutes sont fréquentes. Cependant le diagnostic de rechute est en général plus rapide à faire, puisque la granulomatose de Wegener est déjà connue, et le traitement peut donc être mis en route plus tôt et à stade moins avancé de la maladie.
 

En quoi consiste le traitement de la granulomatose de Wegener?
Des corticoïdes et des immunosuppresseurs sont habituellement prescrits pour obtenir une rémission de la maladie puis limiter le risque de rechute. Le traitement prescrit est fort dans un premier temps(pour induire la rémission), puis les doses des médicaments sont diminuées et les immunosuppresseurs sont changés (allégés) pour maintenir la rémission de façon durable.
L’ensemble du traitement dure un minimum de 18 mois.
 

Qu’est-ce qu’une vascularite?
Une vascularite est définie par l’existence d’une inflammation des vaisseaux sanguins, artères, veines et/ou capillaires. Le groupe des vascularites comporte plusieurs maladies différentes, quimettent parfois le pronostic vital en jeu. Leurs causes sont pour la plupart totalement inconnues, mais avec les progrèseffectués en médecine ces 20 dernières années, des traitements existent aujourd’hui qui ont permis d’en améliorer de façon très significative l’évolution.  La poursuite des recherches biomédicales et thérapeutiques dans le domaine des vascularites reste essentielle pour en améliorer la prise en charge et finalement en guérir.
 

A propos de la Vasculitis Foundation
La Vasculitis Foundation (VF, auparavant Wegener’s Granulomatosis Association) est la plus importante association internationale de soutien et d’information des patients atteints de vascularite et de leur entourage.
 

Les objectifs de la VF, à travers son site internet, ses publications périodiques, ses brochures d’information, ses contacts étroits avec de nombreux chercheurs et médecins spécialistes, ses réunions, ainsi que par la création et le soutien de nombreux groupes de patients à travers le monde, sont d’améliorer la connaissance, d’aider à promouvoir la recherche, et de pouvoir fournir toutes les informations nécessaires, dans le domaine des vascularites, aux patients, à leur famille et à leurs amis pour leur permettre de mieux combattre la maladie.
 

A propos de l’association Wegener Infos Vascularites et du Groupe Français d’Etude des Vascularites
Le Groupe Français d’Etude des Vascularitesest un groupe de recherche thérapeutique et biomédicale créé au début des années 80 en France. Ce groupe participe au conseil scientifique de l’association Française des patients atteints de vascularite, Wegener et Infos Vascularites, créée en Janvier 2006 et qui est le correspondant en France de la VF.

Maladie De Kawasaki

Qu’est-ce que la maladie de Kawasaki?
La maladie de Kawasaki est une maladie très rare qui fait partie du groupe des vascularites systémiques et qui affecte les vaisseaux sanguins de moyen calibre, de façon quasi exclusive chez le très jeune enfant.
 

Quelle est la cause de la maladie de Kawasaki?
On ne connaît pas la cause exacte de cette maladie, mais on sait qu’elle n’est pas directement contagieuse d’un enfant à l’autre. Il existe par contre des périodes dans l’année et des zonesgéographiques dans le monde où la maladie est plus fréquente. On dit souvent que la maladie de Kawasaki est endémo-épidémique, ce qui laisse penser qu’il existe probablement des agents infectieux et/ou de l’environnement à l’origine de la maladie, qui ne sont toujours pas identifiés
aujourd’hui.

Qui sont les personnes atteintes dela maladie de Kawasaki?
Quatre-vingt pourcent des patients atteints de maladie de Kawasaki ont moins de 5 ans.
 

Quels sont les symptômes de la maladie?
Les premiers signes de la maladie sont la fièvre, suivie quelques jours plus tard de lésions des muqueuses (conjonctivite, inflammation des lèvres et de la bouche), de la peau (éruption diffuse sur le corps, ainsi que sur les paumes des mains et des pieds qui deviennentgonflés et oedématiés),associées à une inflammation des ganglions (adénopathies). 

La complication la plus sévère qui peut survenir au cours de cette maladie est liée à l’inflammation des vaisseaux du cœur, c’est-à-dire les artères coronaires. Ces artères peuvent se boucher ou à l’inverse se dilater (anévrisme) et se rompre, exposant au risque d’infarctus du myocarde. La maladie de Kawasaki est aujourd’hui dans les pays industrialisés la première cause d’insuffisance cardiaque acquise de l’enfant.
 

Comment fait-on le diagnostic?
Le diagnostic de la maladie de Kawasaki est avant tout clinique et en général simple à faire. Aujourd’hui, tous les pédiatres connaissent parfaitement cette maladie. Il faut par contre en faire le diagnostic tôt afin de mettre rapidement en route un traitement qui limitera considérablement le risque d’atteinte cardiaque.

Il existe habituellement un syndrome inflammatoire biologique (augmentation de la CRP ou de la vitesse de sédimentation). Le bilan initial doit aussi comporter une radiographie de thorax, un électrocardiogramme et une échographie cardiaque, qui permet de visualiser et d’étudier les artères coronaires chez le petit enfant. Une coronarographie et/ou une artériographie peut/peuvent être nécessaire(s) pour mieux étudier, et parfois aussi pour traiter d’éventuelles lésions vasculaires menaçantes (dilatation des sténoses à l’aide de petits ballonnets gonflables ou pose de stents).
 

Quel est le pronostic de la maladie?
Reconnue et traitée rapidement, une guérison complète et rapide est la règle. Les atteintes cardiaques, lorsqu’elles existent, sont habituellement réversibles sous traitement. Elles restent toutefois à l’origine des 1 à 2% de décès que l’on dénombre encore au cours de cette maladie. Après la guérison, une surveillance cardiologique est indiquée pendant plusieurs années, car une insuffisance cardiaque séquellaire peut parfois se démasquerplus tard dans la vie.
 

Quel est le traitement de la maladie de Kawasaki?
Il existe un traitement spécifique de la maladie de Kawasaki qui doit rapidement être mis en route dès que le diagnostic est fait. Il repose avant tout sur des perfusions à fortes doses d’immunoglobulines intraveineuses et sur la prescription d’aspirine. L’amélioration clinique est habituellement très rapide avec ce traitement, en 24 heures voire moins.
 

Qu’est-ce qu’une vascularite?
Une vascularite est définie par l’existence d’une inflammation des vaisseaux sanguins, artères, veines et/ou capillaires. Le groupe des vascularites comporte plusieurs maladies différentes, qui mettent parfois le pronostic vital en jeu. Leurs causes sont pour la plupart totalement inconnues,
mais avec les progrèseffectués en médecine ces 20 dernières années, des traitements existent aujourd’hui qui ont permis d’en améliorer de façon très significative l’évolution. 

La poursuite des recherches biomédicales et thérapeutiques dans le domaine des vascularites reste essentielle pour en améliorer la prise en charge et finalement en guérir.

A propos de la Vasculitis Foundation
La Vasculitis Foundation (VF, auparavant Wegener’s Granulomatosis Association) est la plus importante association internationale de soutien et d’information des patients atteints de vascularite et de leur entourage. 

Les objectifs de la VF, à travers son site internet, ses publications périodiques, ses brochures d’information, ses contacts étroits avec de nombreux chercheurs et médecins spécialistes, ses réunions, ainsi que par la création et le soutien de nombreux groupes de patients à travers le monde, sont d’améliorer la connaissance, d’aider à promouvoir la recherche, et de pouvoir fournir toutes les informations nécessaires, dans le domaine des vascularites, aux patients, à leur famille et à leurs amis pour leur permettre de mieux combattre la maladie.   

A propos de l’association Wegener Infos Vascularites et du Groupe Français d’Etude des Vascularites 

Le Groupe Français d’Etude des Vascularitesest un groupe de recherche thérapeutique et biomédicale créé au début des années 80 en France. Ce groupe participe au conseil scientifique de l’association Française des patients atteints de vascularite, Wegener Infos et Vascularites, créée en Janvier 2006 et qui est le correspondant en France de la VF.

Periarterite Noueuse

Qu’est-ce que la périartérite noueuse ?

La périartérite noueuse est une maladie très rare qui fait partie du groupe des vascularites systémiques et qui affecte les vaisseaux sanguins de moyen calibre, en particulier ceux de la peau, du cœur, des reins ou du cerveau.

Quelle est la cause de la périartérite noueuse ?

Certains cas de périartérite noueuse sont liés à une infection aiguë par le virus de l’hépatite B (moins de 10% des cas aujourd’hui) ou très rarement par d’autres infections virales (infection par le cytomégalovirus, par exemple). Ces infections virales sont certes transmissibles, mais la périartérite noueuse elle-même n’est pas une maladie contagieuse. Quelques cas de périartérite noueuse ont été décrits en association à une maladie rare du sang, la leucémie à tricholeucocytes. La plupart du temps, cependant, on ne connaît pas la cause de la maladie.

Qui sont les personnes atteintes de périartérite noueuse ?

La périartérite noueuse touche surtout l’adulte âgé de 30 à 50 ans, un peu plus les hommes que les femmes dans certaines études.

Quels sont les symptômes de la maladie ?

Les symptômes de la périartérite noueuse sont variables d’un patient à l’autre et incluent de la fièvre, de la fatigue, un amaigrissement, une perte d’appétit, des douleurs articulaires et musculaires, des éruptions cutanées, des oedèmes des jambes, des ulcérations et/ou des nodules cutanés.

D’autres manifestations peuvent apparaitre, comme des douleurs abdominales ou des saignements digestifs. En cas d’atteinte des nerfs périphériques, les patients peuvent constater une faiblesse musculaire, ressentir des fourmillements ou des douleurs à type de brûlure ou de décharge électrique dans les mains et/ou les pieds. L’atteinte des vaisseaux du cerveau peut entraîner un accident vasculaire cérébral, responsable d’hémiplégie ou de crises convulsives. L’atteinte des artères rénales peut causer une hypertension artérielle sévère et/ou une insuffisance rénale. Si les artères du cœur (les artères coronaires) sont touchées, un infarctus du myocarde, une insuffisance cardiaque avec œdème du poumon ou une péricardite (inflammation de l’enveloppe du cœur) peuvent survenir.

Comment fait-on le diagnostic ?

Il n’existe pas de test spécifique pour faire le diagnostic de périartérite noueuse. C’est devant l’association d’un ensemble de signes cliniques évocateurs et des résultats de plusieurs tests sanguins, radiologiques, et de la biopsie d’un organe ou d’un tissu atteint, que le médecin pourra faire le diagnostic. La plupart des patients ont un syndrome inflammatoire biologique (augmentation du taux de CRP ou de la vitesse de sédimentation). L’angiographie des artères (ou artériographie) digestives et rénales met souvent en évidence des conséquences de l’inflammation des vaisseaux, avec des images de sténoses alternant avec des zones de dilatation (microanévrismes) et parfois des thromboses complètes.

Quel est le pronostic de la maladie ?

Beaucoup de progrès ont été faits ces 30 dernières années dans la prise en charge globale (diagnostic et traitement) de la périartérite noueuse. Sans traitement, la maladie est habituellement mortelle en quelques semaines ou mois. Aujourd’hui, dans la très grande majorité des cas, et lorsque le diagnostic est fait tôt, la maladie peut rapidement être contrôlée et la guérison obtenue. Les rechutes sont rares, mais possibles. Un suivi prolongé est donc nécessaire, même lorsque la maladie a disparu.

Quel est le traitement de la périartérite noueuse ?

Le traitement des formes liées au virus de l’hépatite B repose sur la combinaison de corticoïdes pendant quelques jours ou semaines pour contrôler l’inflammation, d’échanges plasmatiques pour enlever du sang les complexes anticorps-virus qui se sont déposés sur les parois des vaisseaux et ont ainsi causé leur inflammation, et de médicaments antiviraux, comme l’interféron alpha et la lamivudine par exemple, pour débarrasser l’organisme du virus.

Les patients atteints de périartérite noueuse non liée à une infection virale seront traités par l’association de corticoïdes pendant plusieurs mois à fortes doses, auxquels on associera des immunosuppresseurs dans les cas les plus graves.

Qu’est-ce qu’une vascularite ?

Une vascularite est définie par l’existence d’une inflammation des vaisseaux sanguins, artères, veines et/ou capillaires. Le groupe des vascularites comporte plusieurs maladies différentes, qui mettent parfois le pronostic vital en jeu. Leurs causes sont pour la plupart totalement inconnues, mais avec les progrès effectués en médecine ces 20 dernières années, des traitements existent aujourd’hui qui ont permis d’en améliorer de façon très significative l’évolution.

La poursuite des recherches biomédicales et thérapeutiques dans le domaine des vascularites reste essentielle pour en améliorer la prise en charge et finalement en guérir.

A propos de la Vasculitis Foundation

La Vasculitis Foundation (VF, auparavant Wegener’s Granulomatosis Association) est la plus importante association internationale de soutien et d’information des patients atteints de vascularite et de leur entourage.

Les objectifs de la VF, à travers son site internet, ses publications périodiques, ses brochures d’information, ses contacts étroits avec de nombreux chercheurs et médecins spécialistes, ses réunions, ainsi que par la création et le soutien de nombreux groupes de patients à travers le monde, sont d’améliorer la connaissance, d’aider à promouvoir la recherche, et de pouvoir fournir toutes les informations nécessaires, dans le domaine des vascularites, aux patients, à leur famille et à leurs amis pour leur permettre de mieux combattre la maladie.

A propos de l’association Wegener Infos Vascularites et du Groupe Français d’Etude des Vascularites

Le Groupe Français d’Etude des Vascularites est un groupe de recherche thérapeutique et biomédicale créé au début des années 80 en France. Ce groupe participe au conseil scientifique de l’association Française des patients atteints de vascularite, Wegener Infos et Vascularites, créée en Janvier 2006 et qui est le correspondant en France de la VF.

Pseudo-Polyarthrite Rhizomelique

Qu’est-ce que la pseudo-polyarthrite rhizomélique?
La pseudo-polyarthrite rhizomélique (PPR) est un rhumatisme inflammatoire responsable de douleurs et d’une raideurdes épaules et des hanches (ceintures scapulaire et pelvienne), qui peut s'associer avec une artérite temporale giganto-cellulaire (maladie de Horton).

Quelle est la cause de la pseudo-polyarthrite rhizomélique?
On ne connaît pas la cause exacte de cette maladie rhumatismale. On sait par contre qu’elle n’est pas contagieuse. 

Qui sont les personnes atteintes de pseudo-polyarthrite rhizomélique?
Des signes de pseudo-polyarthrite rhizomélique sont observés chez environ 50% des patients atteints de maladie de Horton. A l’inverse, on estime que 15% des patients atteints de pseudo-polyarthrite rhizomélique risque d’avoir ensuite une maladie de Horton. Il existe probablement un terrain de susceptibilité génétique commun à ces 2 maladies. La pseudo-polyarthrite rhizomélique survient, comme la maladie de Horton, surtout chez des personnes âgées de plus de 50 ans.
 

Quels sont les symptômes de la maladie?
Les symptômes les plus classiques de la pseudo-polyarthrite rhizomélique sont des douleurs et une raideur matinale des épaules et/ou des hanches, en général des 2 côtés, mais parfois de façon non symétrique. Il existe aussi parfois des douleurs cervicales et/ou du dos.
 

D’autres signes peuvent être présents comme une intense fatigue générale, un amaigrissement et/ou de la fièvre. Certains patients ont des œdèmes ou un gonflement des chevilles, des pieds, des poignets et/ou des mains.
 

Il faut aussi rechercher des signes en faveur d’une artérite giganto-cellulaire associée, comme des maux de tête, des douleurs des mâchoires ou des troubles récents de la vue.
 

Comment fait-on le diagnostic?
Le diagnostic de pseudo-polyarthrite rhizomélique est clinique. Il n’existe pas de test spécifique de cette maladie. Il existe par contre habituellement un syndrome inflammatoire biologique, avec augmentation du taux de CRP et de la vitesse de sédimentation (VS).
 

Une biopsie d’artère temporale peut être demandée par le médecin s’il existe des signes cliniques pouvant correspondre à une artérite giganto-cellulaire associée.
 

Quel est le pronostic de la maladie?
La pseudo-polyarthrite rhizomélique peut parfois guérir spontanément et sans traitement en 1 à 4 ans. Les manifestations cliniques douloureuses imposent cependant souvent un traitement,  qui est la plupart du temps extrêmement et très rapidement efficace. Ce traitement repose les corticoïdes, prescrits pendant 1 à 2 ans.
 

Quel est le traitement de la pseudo-polyarthrite rhizomélique?
Le traitement est une corticothérapie prolongée, débutée à des doses relativement importantes et adaptées au poids du patient. L’amélioration clinique et la régression du syndrome inflammatoire biologique sont en général très rapidement obtenues, et les doses de corticoïdes peuvent être en général êtreprogressivement diminuées dès la 2 ème à 4 ème semaine. Lescorticoïdesdoivent ensuite être maintenus, à faibles doses, pendant plusieurs mois pour éviter des rechutes précoces, constatées chez 25–50% des patients.
 

Qu’est-ce qu’une vascularite?
Une vascularite est définie par l’existence d’une inflammation des vaisseaux sanguins, artères, veines et/ou capillaires. Le groupe des vascularites comporte plusieurs maladies différentes, quimettent parfois le pronostic vital en jeu. Leurs causes sont pour la plupart totalement inconnues, mais avec les progrèseffectués en médecine ces 20 dernières années, des traitements existent aujourd’hui qui ont permis d’en améliorer de façon très significative l’évolution.
 

La poursuite des recherches biomédicales et thérapeutiques dans le domaine des vascularites reste essentielle pour en améliorer la prise en charge et finalement en guérir.
 

A propos de la Vasculitis Foundation
La Vasculitis Foundation (VF, auparavant Wegener’s Granulomatosis Association) est la plus importante association internationale de soutien et d’information des patients atteints de vascularite et de leur entourage.
 

Les objectifs de la VF, à travers son site internet, ses publications périodiques, ses brochures d’information, ses contacts étroits avec de nombreux chercheurs et médecins spécialistes, ses réunions, ainsi que par la création et le soutien de nombreux groupes de patients à travers le monde, sont d’améliorer la connaissance, d’aider à promouvoir la recherche, et de pouvoir fournir toutes les informations nécessaires, dans le domaine des vascularites, aux patients, à leur famille et à leurs amis pour leur permettre de mieux combattre la maladie.
 

A propos de l’association Wegener Infos Vascularites et du Groupe Français d’Etude des Vascularites
Le Groupe Français d’Etude des Vascularitesest un groupe de recherche thérapeutique et biomédicale créé au début des années 80 en France. Ce groupe participe au conseil scientifique de l’association Française des patients atteints de vascularite, Wegener Infos et Vascularites, créée en Janvier 2006 et qui est le correspondant en France de la VF.

Syndrome De Churg Et Strauss

Qu’est-ce que le syndrome de Churg et Strauss ?

Le syndrome de Churg et Strauss est une maladie très rare qui fait partie du groupe des vascularites systémiques et qui affecte les vaisseaux sanguins de petit et moyen calibres. Cette maladie se caractérise, par rapport aux autres vascularites, par l’existence d’un asthme, survenant tardivement dans la vie, de façon surprenante, ou qui est plus ancien et s’aggrave de façon inexpliquée. Il s’y associe habituellement une augmentation dans le sang du nombre d’un certain type de globules blancs, les polynucléaires éosinophiles.

Le syndrome de Churg et Strauss évolue de façon schématique en 3 phases successives : une première phase dite « allergique » (asthme, rhinite allergique), une seconde dite « hyperéosinophilique » (augmentation des polynucléaires éosinophiles dans le sang) et enfin une phase de « vascularite » à proprement parler. Tous les patients ne passent cependant pas aussi schématiquement par chacune des ces 3 phases.

En l’absence de traitement, le syndrome de Churg et Strauss peut toucher n’importe quel organe du corps, notamment les poumons, le cœur, les nerfs, la peau, le tube digestif, les reins, les yeux, les muscles, les articulations et, plus rarement, le cerveau.

Quelle est la cause du syndrome de Churg et Strauss ?

On ne connaît pas la cause exacte de cette maladie, et il n’y en a probablement pas qu’une seule. On sait par contre qu’elle n’est pas contagieuse.

Qui sont les personnes atteintes de syndrome de Churg et Strauss ?

Le syndrome de Churg et Strauss peut survenir à tout âge. Cependant, elle se révèle surtout chez l’adulte âgé de 35 à 45 ans, aussi bien homme que femme.

Quels sont les symptômes de la maladie ?

Les symptômes et leur nombre sont très variables d’un patient à l’autre, en fonction des organes atteints et du stade évolutif de la maladie. Durant les premières phases de la maladie, la grande majorité des patients souffrent d’asthme, dont le contrôle est difficile malgré la prescription de différents traitements inhalés (en spray). Par la suite, on peut observer un amaigrissement, une fatigue générale, de la fièvre, une toux, des éruptions cutanées urticariennes, une sinusite ou de douleurs sinusiennes. Ces symptômes peuvent persister ou s’aggraver durant la phase de constitution de la vascularite, de même que des douleurs abdominales, l’émission de sang dans les selles, un essoufflement au moindre effort, des douleurs dans la poitrine, dans les muscles et les articulations, des impressions de fourmillements ou d’anesthésie dans les mains ou les jambes, des troubles visuels et/ou une confusion mentale.

Comment fait-on le diagnostic ?

Le diagnostic de syndrome de Churg et Strauss est difficile et ne peut souvent être fait que plusieurs semaines ou mois après l’apparition des premiers symptômes. Ceci s’explique par l’extrême rareté de la maladie, alors que la maladie asthmatique est très fréquente, par la grande variabilité des signes cliniques d’un individu à l’autre, et par l’absence de test biologique spécifique. La combinaison, chez un même patient, d’un asthme rebelle, d’une hyperéosinophilie sanguine et d’antécédents d’allergies ou de problèmes de sinus doit certes faire évoquer le diagnostic, mais cela ne suffit pas. Le diagnostic doit dans l’idéal être confirmé par la biopsie d’un tissu ou d’un organe atteint, notamment des poumons.

Des auto-anticorps dirigés contre le cytoplasme des polynucléaires neutrophiles (les ANCA) peuvent être détectés dans le sang de 35 à 50% des patients.

Quel est le pronostic de la maladie ?

Dans les études publiées, plus de 80% des patients, traités de façon adaptée, sont vivants 5 ans après le diagnostic (c’est ce qu’on appelle le taux de survie à 5 ans). La rémission est obtenue chez la plupart des patients, même si de petites rechutes peuvent survenir par la suite. Un suivi prolongé par des médecins spécialistes est donc nécessaire.

Quel est le traitement du syndrome de Churg et Strauss ?

Durant les premières phases de la maladie, si le diagnostic est fait tôt et qu’il n’y a pas d’atteinte des organes vitaux (cœur, rein et/ou cerveau notamment), un traitement par corticoïdes seuls peut être suffisant. Par contre, si des organes vitaux sont touchés, des immunosuppresseurs (en particulier le cyclophosphamide) devront être prescrits en association aux corticoïdes.

Qu’est-ce qu’une vascularite ?

Une vascularite est définie par l’existence d’une inflammation des vaisseaux sanguins, artères, veines et/ou capillaires. Le groupe des vascularites comporte plusieurs maladies différentes, qui mettent parfois le pronostic vital en jeu. Leurs causes sont pour la plupart totalement inconnues, mais avec les progrès effectués en médecine ces 20 dernières années, des traitements existent aujourd’hui qui ont permis d’en améliorer de façon très significative l’évolution.

La poursuite des recherches biomédicales et thérapeutiques dans le domaine des vascularites reste essentielle pour en améliorer la prise en charge et finalement en guérir.

A propos de la Vasculitis Foundation

La Vasculitis Foundation (VF, auparavant Wegener’s Granulomatosis Association) est la plus importante association internationale de soutien et d’information des patients atteints de vascularite et de leur entourage.

Les objectifs de la VF, à travers son site internet, ses publications périodiques, ses brochures d’information, ses contacts étroits avec de nombreux chercheurs et médecins spécialistes, ses réunions, ainsi que par la création et le soutien de nombreux groupes de patients à travers le monde, sont d’améliorer la connaissance, d’aider à promouvoir la recherche, et de pouvoir fournir toutes les informations nécessaires, dans le domaine des vascularites, aux patients, à leur famille et à leurs amis pour leur permettre de mieux combattre la maladie.

A propos de l’association Wegener Infos Vascularites et du Groupe Français d’Etude des Vascularites

Le Groupe Français d’Etude des Vascularites est un groupe de recherche thérapeutique et biomédicale créé au début des années 80 en France. Ce groupe participe au conseil scientifique de l’association Française des patients atteints de vascularite, Wegener Infos et Vascularites, créée en Janvier 2006 et qui est le correspondant en France de la VF.

Vascularite De La Polyarthrite Rhumatoide

Qu’est-ce que la vascularite de la polyarthrite rhumatoïde?
La polyarthrite rhumatoïde est une maladie rhumatisme inflammatoire chronique qui peut, dans de rares cas, se compliquer de vascularite, lorsque l’inflammation touche aussi les vaisseaux sanguins de petit et/ou moyen calibre(s). On parle parfois de vascularite rhumatoïde, qu’il ne faut pas confondre avec le purpura rhumatoïde, qui est une autre maladie touchant surtout le jeune enfant et qui faitaussipartie du groupe des vascularites systémiques.
 

Quelle est la cause de la polyarthrite rhumatoïde?
On ne connaît pas la cause exacte de ce rhumatisme inflammatoire chronique. On sait par contre que ce n’est pas contagieux. Il existe probablement un emballement du système immunitaire des patients qui s’attaque alors par erreur à leurspropres articulations et, en cas de vascularite,à leurs propres vaisseaux sanguins (on parle de maladie auto-immune).
 

Qui sont les personnes atteintes de vascularite de la polyarthrite rhumatoïde?
Par définition, la vascularite de la polyarthrite rhumatoïde touche des individus atteints de polyarthrite rhumatoïde. Les patients qui font cette complication rare ont habituellement une polyarthrite rhumatoïdeévoluant depuis plusieurs années et qui reste active malgré des traitements spécifiques. Ils ont souventaussi un taux élevé de facteur rhumatoïde dans le sang (test utile au diagnostic de polyarthrite rhumatoïde). Les hommes semblent avoirun plus de risque de développer cette complication.
 

Quels sont les symptômes de la maladie?
La vascularite de la polyarthrite rhumatoïde peut affecter n’importe quel organe ou tissu du corps, en particulier la peau, les nerfs des mains et/ou pieds, et les petits vaisseaux des doigts et/ou orteils. L’atteinte cutanée est la plus fréquente des manifestations (90% des patients), et se traduit le plus souvent par des petits points rouges sur la peau (lésions ditespurpuriques). Les yeux peuvent aussi être touchés, avec des aspects d’œil rouge douloureux (sclérite) ou par l’installation d’un flou visuel par inflammation des liquides de la partie antérieure de l’œil (uvéite).

Le cœur peut aussi être atteint, avec une inflammation de son enveloppe (péricardite) ou des troubles du rythme (arythmie). Le risque le plus important reste celui d’infarctus du myocarde.
 

Les patients ayant une polyarthrite rhumatoïde sont suivis régulièrement durant toute leur vie, et doivent donc consulter en cas d’apparition de symptômes inhabituels comme un amaigrissement, de la fièvre, une fatigue générale, des douleurs thoraciques, des troubles de la sensibilité desmains et/oupieds, des lésions cutanées ou tout autre symptôme extra-articulaire.
 

Comment fait-on le diagnostic?
Savoir que le patient est atteint de polyarthrite rhumatoïde ne suffit pas pour faire le diagnostic de cette complication qu’est la vascularite rhumatoïde. En pratique, une biopsie d’un tissu atteint est presque toujours nécessaire pour confirmer ce diagnostic. Il peut s’agir d’une biopsie de nerf, de peau ou de rein, en fonction des atteintes observées. Parfois, des vaisseaux de plus gros calibre peuvent être touchés et une angiographie sera alors réalisée pour étudier l’apparence des vaisseaux atteints et localiser les lésions les plus importantes.
 

Quel est le pronostic de la maladie?
Peu d’études sont disponibles permettant de connaître l’évolution générale des patients atteints de vascularite de la polyarthrite rhumatoïde. Il s’agit en effet d’une complication très rare de ce rhumatisme inflammatoire, qui, lui, est par contre très fréquent. Tout ce qu’on peut dire, c’est que chez les patients atteints de polyarthrite rhumatoïde, le pronostic global de ceux atteints de vascularite rhumatoïde semble un peu moins bon que ceux n’ayant pas cette complication, ce qui semble assezlogique.

Quel est le traitement de la vascularite de la polyarthrite rhumatoïde?
En cas de confirmation diagnostique de vascularite rhumatoïde, un traitement agressif doit être prescrit. Des corticoïdes sont toujours nécessaires, en association à d’autres médicaments pour contrôler la polyarthrite elle-même, et parfois à d’autres immunosuppresseurs encore plus puissants.
 

Qu’est-ce qu’une vascularite?
Une vascularite est définie par l’existence d’une inflammation des vaisseaux sanguins, artères, veines et/ou capillaires. Le groupe des vascularites comporte plusieurs maladies différentes, quimettent parfois le pronostic vital en jeu. Leurs causes sont pour la plupart totalement inconnues, mais avec les progrèseffectués en médecine ces 20 dernières années, des traitements existent aujourd’hui qui ont permis d’en améliorer de façon très significative l’évolution. 

La poursuite des recherches biomédicales et thérapeutiques dans le domaine des vascularites reste essentielle pour en améliorer la prise en charge et finalement en guérir.
 

A propos de la Vasculitis Foundation
La Vasculitis Foundation (VF, auparavant Wegener’s Granulomatosis Association) est la plus importante association internationale de soutien et d’information des patients atteints de vascularite et de leur entourage.

Les objectifs de la VF, à travers son site internet, ses publications périodiques, ses brochures d’information, ses contacts étroits avec de nombreux chercheurs et médecins spécialistes, ses réunions, ainsi que par la création et le soutien de nombreux groupes de patients à travers le monde, sont d’améliorer la connaissance, d’aider à promouvoir la recherche, et de pouvoir fournir toutes les informations nécessaires, dans le domaine des vascularites, aux patients, à leur famille et à leurs amis pour leur permettre de mieux combattre la maladie.
 

A propos de l’association Wegener Infos Vascularites et du Groupe Français d’Etude des Vascularites
Le Groupe Français d’Etude des Vascularitesest un groupe de recherche thérapeutique et biomédicale créé au début des années 80 en France. Ce groupe participe au conseil scientifique de l’association Française des patients atteints de vascularite, Wegener Infos et Vascularites, créée en Janvier 2006 et qui est le correspondant en France de la VF.

Do Enzymes Called Matrix Metalloproteinases Play a Role in GCA? - Alicia Rodriguez-Pla, M.D., Ph.D.

Phillip Seo, M.D., MHS

Assistant Professor of Medicine

The Johns Hopkins University School of Medicine

Division of Rheumatology

Co-Director, The Johns Hopkins Vasculitis Center

Vasculitis Clinical Research Consortium Co-Investigator

It is a great pleasure to introduce Dr. Rodriguez-Pla to the members of the VF community. Dr. Rodriguez-Pla was recruited to the Johns Hopkins Division of Rheumatology by Dr. John Stone, who was the Director of the Vasculitis Center at that time. Her research interests have focused on the etiopathogenesis of giant cell arteritis, which is the most common form of primary systemic vasculitis in the United States.                       

In collaboration with Wills Eye Hospital in Philadelphia, she has been given access to multiple preserved temporal artery specimens obtained from patients suspected of having giant cell arteritis. These specimens served as the basis of an early project looking for evidence of an infectious etiology for this disease.                       

Dr. Rodriguez-Pla’s more recent research has examined the role played by matrix metalloproteinases in the development of giant cell arteritis.  This work was presented at the American College of Rheumatology Annual Scientific Meeting in Washington, D.C. last year. Her future research efforts will undoubtedly do much to further our understanding of these complex and challenging diseases.

By Alicia Rodriguez-Pla, M.D., Ph.D., March 2007  

I grew up in the southeast of Spain, between Caudete, a small town in which I was born, and Alicante, a nearby Mediterranean tourist city. I am married and have a four-month-old baby son called Mark. My husband, José, is also a doctor who specializes in Preventive Medicine and Public Health. 

I spend as much of my free time as possible with my husband and playing with my son. I like travelling and meeting people from different cultures. I love reading all kinds of books, especially novels, walking, swimming, going to the theater and getting together with friends.  Although it may sound weird, studying and learning are my main hobbies.

I realized I wanted to become a doctor in high school. During those years I started to develop a great interest in health related issues, primarily for helping people and for research, which I consider the basic tool for medical advances.   And last but not least, decreasing the negative impact of diseases in people. 

I received a scholarship which allowed me to attend medical school in a very prestigious private university called Universidad de Navarra, in Pamplona, a city in the north of Spain. After medical school I moved to Barcelona and enrolled in a fellowship program in rheumatology. Once completed, I worked on my Ph.D. thesis, where I studied several pathogenic and epidemiologic aspects of giant cell arteritis. It was shortly after defending my thesis in 2003 that I moved to the United States.

Very early in my career I became fascinated by vasculitis disorders. My first clinical rotation was in the Department of Cardiovascular Surgery in 1991, during my fourth year in  medical school. I remember the first patient I visited in the hospital was a woman affected by Takayasu’s arteritis who had also developed retroperitoneal fibrosis. Never before had I heard that blood vessels could become inflamed. The faculty in charge asked me to review the literature to find out if a similar association between Takayasu’s arteritis and retroperitoneal fibrosis had previously been reported. I found only one case published in a Japanese journal, which was impossible for me to get at the time. The faculty then asked me to write a short review about Takayasu’s arteritis as an exercise. I remember how my interest and curiosity progressively increased while reviewing the literature and learning about this vasculitis. I typed a thirteen-page manuscript, which I still keep, on a classical typewriter. It was because of the interest that this patient woke up in me that I decided to become a rheumatologist.  

I did not come across any other patients with vasculitis until January 1995, when I saw a second patient with Takayasu’s arteritis during my first month as a fellow in rheumatology. This was striking, taking into consideration that this type of vasculitis is quite rare in Spain. During the following months I saw patients with other vasculitic syndromes, including Wegener’s granulomatosis, Churg Strauss Syndrome, polyarteritis nodosa, and giant cell arteritis among others.  I was amazed that a patient with giant cell arteritis, who had been completely blind for two days, recovered her vision right after starting prednisone treatment. Fortunately, she had been diagnosed and treated early.  

As a curiosity, my father was the third patient I saw with giant cell arteritis and I participated actively in his diagnosis. In May 1996, at the age of 53, he started complaining of intense headaches, fatigue, and pain and weakness in the girdles. He was initially diagnosed with depression, and treated for several months with antidepressant medication. He did not improve, and I suggested to his doctor the possibility of giant cell arteritis. Initially, he did not believe me because my father was too young to have this vasculitis, and I was too young to suggest anything. After almost six months, he finally decided to draw some blood, and the diagnosis then became quite obvious.   

I would like to point out that my interest comprises all kinds of vasculitic syndromes and all vasculitis patients. I chose giant cell arteritis for my Ph.D. thesis simply because it is the most frequent type of vasculitis worldwide including Spain.   I am convinced that the pathogenic mechanisms underlying the vascular and organ damage have certain similarities among the different vasculitic disorders, and that advances in knowledge of one of them could be quite easily extrapolated to others. 

My Ph.D. thesis included a project to study the potential role of different infectious agents in giant cell arteritis. At the American College of Rheumatology Annual Scientific Meeting in San Francisco in 2001, I saw that the group of physicians, including Dr. Stone from the Johns Hopkins Vasculitis Center were also interested in this field. Shortly after the meeting, I contacted Dr. Stone to ask about the possibility of joining his team to continue working on this research.  Dr. Stone offered me a two-year fellowship at Johns Hopkins. Towards the end of these two years, my research interest had shifted and focused on understanding the molecular mechanisms underlying the vascular damage in the arterial wall in giant cell arteritis. It was at this point when the VCRC fellowship program was starting, and Dr. Stone mentored me to get into it.

My research is currently focused on better understanding the role of matrix metalloproteinases in giant cell arteritis. Matrix metalloproteinases are a group of enzymes capable of degrading varying components of the extracellular matrix in both normal and diseased tissue. They have been found to be associated with several vasculitic disorders. In concrete, I am working on two different laboratory projects. The first consists on characterizing the specific matrix metalloproteinases involved in vascular damage in giant cell arteritis. The second is a genetic study to determine if certain polymorphisms, that is, mutations, of metalloproteinase-9, a enzyme known to be involved in giant cell arteritis, are associated with this vasculitis.

One of the main things I have learned from my patients is to listen to them.  Listening carefully to patients’ symptoms helps us to suspect a serious condition or relapse. Therefore we may act quickly avoiding delays in treatment and, subsequently, preventing or diminishing complications.

I think that to be a better patient is essential to follow the physician’s indications. Of course, this should not be done “blindly” but rather in a “educated” manner. By this I mean that if a patient finds, for example, that a treatment is not working, call the doctor to discuss it with him/her. Another aspect would be by understanding that research is very important for advancing knowledge of the disease, and for developing new therapeutic options. An important challenge for doing investigation on vasculitis is the rarity of these disorders, which makes it difficult to get clinical samples for research purposes. By agreeing to participate in research projects whenever possible, patients can help to get better outcomes for themselves and others.Because most of the patients with vasculitis have chronic conditions, which require long-term follow-up, it is extremely important for them to engage in a good doctor-patient relationship. It is basic for them to find a physician they truly trust, and with whom they can communicate in a direct, open and honest way. They should take good care of themselves, attend regular follow-ups, and learn as much as they can about their diseases. The latter will help them to recognize promptly when they could be suffering from a relapse, which will lead them to look for the best medical care in every situation.

My main job duty is to do research in the laboratory setting. I am also a student in the part-time Master of Public Health program at the Johns Hopkins Bloomberg School of Public Health.  So far, I have taken a series of courses in statistics and epidemiology, along with other required courses. I am currently taking a third series of courses on Genetic Epidemiology.  I plan to graduate next May.

In addition, I have also been allowed to observe patients in The Johns Hopkins Vasculitis Center. During my stay at Johns Hopkins I have seen more patients affected by Wegener’s granulomatosis, Churg Strauss syndrome, polyarteritis nodosa, and Takayasu’s arteritis than ever before.  Unfortunately, for the last few months my lab work and classes in Public Health have made it impossible for me to attend the clinic. 

I am very grateful to the VCRC for the opportunity it has provided me through this fellowship. My weekly visits to The Johns Hopkins Vasculitis Center clinic have offered me a unique opportunity to see a great variety of vasculitis patients, and some of them while going through challenging medical situations. This has greatly enhanced my clinical skills to manage patients with vasculitis. This fellowship has also helped me to increase my laboratory skills, and it has provided me with the opportunity of enrolling in an academic program in the form of a Master of Public Health in the worldwide prestigious Johns Hopkins Bloomberg School of Public Health. In addition, from the personal point of view, this experience has been also very fulfilling.

During my time at Johns Hopkins I would like to increase both my skills as a translational researcher and as a clinician. I think that the tridimensional approach of the training program I have been involved in during my stay here will help me to accomplish this goal. My bench work and the courses of the Master of Public Health program are providing me with essential tools to design and perform translational and epidemiologic investigations. In addition, the time I have spent in clinic has helped me to be a better and more skilled physician for my patients. By combining this training program, the detailed “picture” offered by the laboratory bench research, the patients observation in the clinical setting and the big “picture” provided by the epidemiology and public health perspective, I believe I can integrate a broad range of knowledge, that will overall and help me to be a better scientist.

Once I leave Johns Hopkins, I would like to continue working on vasculitis, and probably on other autoimmune and inflammatory disorders in Spain. I hope the skills I have acquired during my stay here will help me to earn a faculty position in Spain in where I can combine both, patient care and research. I would also like to participate in international projects in the future, which include collaboration among different countries. 

Francisco Silva, M.D. - Involved in Numerous Research Projects Hones Skills at Mayo

Ulrich Specks, M.D.

Professor of Medicine

Division of Pulmonary & Critical Care Medicine

Mayo Clinic

Rochester, MN 55905

Dr. Silva came to the Mayo Clinic in August of 2006 as a Vasculitis Clinical Research Fellow.  His fellowship is supported by the NIH-Rare Diseases Network and the multicenter Vasculitis Clinical Research Consortium.  The fellowship is designed as a post graduate training program that provides two additional years of post-graduate training in vasculitis research to rheumtologists, nephrologists, pulmonologists or other sub-specialists.  During this time he will achieve a Masters Degree in Clinical Research and complete several translational and clinical research projects.

Dr. Silva’s work at Mayo Clinic focuses on the identification of specific PR3-ANCA subsets that recognize specific epitopes, and he is investigating their utility as biomarkers of disease activity using samples from large trial cohorts.  In addition, he is involved in the VCRC longitudinal cohort studies and in a follow-up study of the Wegener’s granulomatosis etanercept trial.  Furthermore, he is investigating the clinical outcome of patients with orbital involvement of Wegener’s granulomatosis and the efficacy of mycophenolate mofetil in MPO-ANCA positive microscopic polyangiitis with mild renal involvement.

After completion of the VCRC fellowship and master’s degree program, Dr. Silva will return to Santiago, Chile, where he plans to establish a vasculitis center and conduct clinical research studies.

Since his arrival here last fall, I have gotten to know Dr. Silva’s enthusiasm and dedication to vasculitis and research.  He has picked up laboratory methods as quickly as the important theoretic pathogenetic concepts.  He is very inquisitive and extremely productive, and his work has already generated data for several abstract presentations and research papers.  But most importantly, through his caring personality he has enriched all of us who have had the privilege to meet Dr. Silva and work with him.  I have no doubt that Dr. Silva will succeed with his Master’s Degree program here at Mayo Clinic, but also with his long-term quest to become an independent clinician-investigator upon his return to Santiago, and I am looking forward to his continued long term collaborations with Mayo Clinic and the VCRC.

by Francisco Silva, M.D.

I grew up in Santiago, Chile where I completed both my undergraduate and medical degrees at the Catholic University of Chile.  I am married to Alejandra Araya who is a nurse and is completing her Ph.D. in Nursing Science at the University of Illinois in Chicago.  I enjoy reading Astronomy and observing the night sky.  I have especially enjoyed seeing the constellations from the northern hemisphere.

I became interested in becoming a physician in high school where I had the opportunity to participate in a scientific group organized by the Biology teacher.  It was a fantastic period in which I discovered the wonderful world of the microscope, fossils and experiments.  Since that time my interests in biology and the human body led me to medicine as the vocation that fulfills both.

Patients with vasculitis have fascinated me because of the complex nature of their disease manifestation. They are the type of patients you never forget.  To improve their treatment and care, we need to answer many open questions about etiology, pathophysiology and their relationship to other autoimmune diseases.

My interest in vasculitis was sparked by one most memorable patient I took care of during my early days of training.  She was a delightful woman in a public hospital in Chile who presented with skin ulcers on her legs.  This woman had histologically confirmed microscopic polyangiitis and a positive P-ANCA.  What initially seemed to be a minor ulcer progressed rapidly to compromise the entire foot, and new ulcers appeared on her upper extremities.  She also developed renal insufficiency.  I was impressed that a disease that initially presented itself as mild could progress so rapidly to extensive multi-system involvement.   After months of therapy she recovered physically and emotionally, more so than I ever expected. I was extremely impressed by her level of resilience.

My patients teach me about their lives and their disease – this is important, as it is a useful guide for the research I am involved in.  I try to teach vasculitis patients that they need to face their disease head-on and need to adhere to therapy.  They need to listen to what their bodies tell them so that they can work to keep their disease under control.

I am most grateful to the VCRC for providing me with the opportunity to improve my translational and clinical research skills through the fellowship at Mayo Clinic.  I am pursuing a Masters Degree in Clinical Research, and I am involved in several translational research studies.  These focus on the utility of PR3-ANCA subsets as biomarkers for disease activity, organ manifestations and prognosis in Wegener’s granulomatosis, and on the long term outcome of different therapies in the ANCA-associated vasculitides.

During my time at the Mayo Clinic I would like to acquire the knowledge and skills needed to become an independent translational clinician investigator. I think the Mayo Clinic is an ideal place to achieve this goal.  When I have completed my work here I want to return to my country, to become an expert in the care of vasculitis patients and to develop translational research projects in vasculitis.

Irish CCF Fellow Specializles in Patient Care and Vasculitic Investigation - Eamonn Molloy, M.D.

Carol A. Langford, M.D., MHS

Director, Center for Vasculitis Care and Research

Cleveland Clinic

I am pleased to introduce Dr. Eamonn Molloy who has been with us at the Cleveland Clinic since August, 2005 and who formally began his vasculitis fellowship in July, 2006. Thanks to the leadership of Dr. Gary Hoffman, the training of physicians interested in vasculitis has had a long history in the Cleveland Clinic Center for Vasculitis Care and Research. During their training, these physicians have contributed meaningful scientific work that has enhanced our understanding of vasculitis. After completing their fellowships, our graduates have provided expert care to vasculitis patients in Mexico, Asia, South America, Canada and the USA.

Even though our own Institution's enthusiasm and resources have been very important to our fellowship program, the training of a vasculitis expert is not possible without funding. The Vasculitis Clinical Research Consortium (VCRC) Fellowship that was discussed in an earlier Vasculitis Foundation newsletter is an important landmark in funding vasculitis education. However, it will be a long time before we have adequate numbers of vasculitis experts. Patient needs are often unmet and we lack a critical number of talented physician investigators who can lead future research directed towards finding cures. It is for this reason that we are so deeply appreciative of the Vasculitis Foundation's support of fellowship training.

Dr. Molloy is an excellent example of the promise of such training. As seen in his comments, Dr. Molloy is engaging and sincere in his commitment to vasculitis. His excellent education to date earned him funding from the R.J. Fasenmyer Center for Clinical Immunology to partially support his quest to acquire advanced knowledge and skills. However, if it was not for the generous support of the Vasculitis Foundation, completion of his vasculitis fellowship and Masters Degree studies would not be possible. It has been a great pleasure for all of us in Cleveland to work with Dr. Molloy and we are tremendously grateful for the support of the Vasculitis Foundation which has invested in our common goal to help people with vasculitis. 

By Eamonn Molloy, M.D., September 2006 

I was born and raised in the western part of Ireland. As a family we were heavily involved with Irish culture – language, music and sports. There is no medical background in my family; my boyhood dreams focused more on winning the All-Ireland Gaelic Football championship with my county team, but the likelihood of those dreams coming to fruition was critically undermined by a lack of sporting prowess!

I first considered a career in medicine at age 17. The Irish system of medical training differs substantially from that in the United States, requiring the completion of a six year course, which is generally pursued directly after finishing secondary school (the equivalent of high school). The system for college entry is based on the ‘Leaving Certificate’, an examination taken at the end of secondary school. This is the most onerous examination that Irish students face at any time during their academic career, with approximately 10 days of examinations, typically consisting of 6 hours of written/essay papers per day across at least 6-7 subjects.

My decision to apply for medical school was a late one, coming after completion of the Leaving Certificate, just before the deadline for revised submission of college course choices. I had not been sure as to what I wanted to do in college because I enjoyed studying a broad range of subjects at secondary level. I had not really considered medicine until my mother suggested it while I was discussing with her some of the latest medical news from Time magazine. The more I thought about it the more it appealed to me so I applied about one week later, started medical school at University College Dublin in October 1991 and I have not regretted it since.

The six year medicine course is followed by a one year internship (six months of internal medicine and six months of surgery). At this point, doctors are fully registered practitioners. Subsequent to this, I entered a senior house officer scheme (analogous to a residency scheme) in internal medicine at the Mater Misericordiae University Hospital (MMUH) in Dublin. This provided a number of key experiences that influenced my subsequent career choices. A 3-month rotation on the Rheumatology service convinced me that my future lay within this specialty. The patient-centered approach and the potential to build a rapport with patients over the course of their chronic illness was an important factor in this decision. Many of the rheumatic conditions are multi-system diseases that provide a diagnostic and therapeutic challenge for the clinician. Even at that early stage, I was particularly stimulated by the challenge provided by the vasculitis patients that I cared for during this rotation.

As part of the MMUH Senior House Officer scheme, I then spent a six month period as an internal medicine resident at the Mayo Clinic, Rochester, Minnesota, USA. This was another important experience, as it emphasized the potential benefits to my career of seeking specialty training in a top-level academic center in the USA.

I commenced the Specialist Registrar (SpR) program in Rheumatology and Internal Medicine in July 2000. This is a 5-year program, overseen by the Royal College of Physicians in Ireland, which ultimately leads to Certification of Satisfactory Completion of Specialist Training (CSCST; analogous to specialty board certification in the USA). The first three years of my SpR program were spent in internal medicine and rheumatology at three different teaching hospitals in Ireland, including one year at MMUH, where one of my mentors was Dr. Geraldine McCarthy. I subsequently began a two-year research experience under the supervision of Dr. McCarthy. This was undertaken at the Department of Molecular and Cellular Therapeutics at the Royal College of Surgeons of Ireland Medical School in Dublin and MMUH. This laboratory-based research explored the role of prostaglandins in calcium crystal-associated osteoarthritis. Based on this work I submitted a thesis for a Doctorate of Medicine degree through the National University of Ireland, which was accepted in January 2006.

Health care in Ireland is provided mainly by a public health care system, supplemented by a number of private hospitals. The government-funded public health care system provides acute and elective care for the entire population. The private hospitals mainly engage in elective clinic services and non-acute inpatient care. Because of the shortcomings in the public system, approximately half of the population purchase private health insurance so that they can access these services in the private sector in a more timely fashion.

Rheumatology has historically been underserved in Ireland, with long waiting times for clinic appointments in many centers. Vasculitis has a relatively low profile in Ireland, with no established vasculitis center. Because of the shortage of rheumatology posts, demand for positions and quality of applicants has been high. Therefore, virtually all rheumatologists have trained both in Ireland and in international academic centers, mainly in the United Kingdom and North America, and have thereby gained significant experience in management of patients with complex rheumatic diseases. However, this scarce resource is further diminished by the fact that the vast majority practice as dual internists-rheumatologists. A new strategy aims to considerably increase the number of rheumatologists, and critically, also the number of appointments in the allied health professions. Hopefully, if implemented, this strategy will significantly increase the resources available to Irish patients with vasculitis and other rheumatic diseases.

As mentioned above, I have had a specific interest in vasculitis from an early point in my career. To further my goal of sub-specialty training in vasculitis, I sought out the opportunity to participate in the Vasculitis Fellowship Program at the Center for Vasculitis Care and Research at the Department of Rheumatic and Immunologic Diseases, Cleveland Clinic Foundation. This commenced in August 2005. Since then I have gained invaluable experience in the care of vasculitis patients under the supervision of Dr. Carol Langford (Program Director), Dr. Gary Hoffman (Department Chairman) and Dr. Leonard Calabrese.  I am planning to spend a further two years as a vasculitis fellow at the Cleveland Clinic Foundation, to gain further experience in the care of patients with these complex diseases. In addition, I have enrolled in the two-year Clinical Research Scholars Program at Case Western Reserve University. This program is specifically aimed at teaching clinical investigative skills, culminating in the award of a Master of Science in Clinical Research degree.

The support provided by the Vasculitis Foundation Fellowship will enable me to achieve these goals. My ultimate objective is to develop the skills necessary to provide expert care to vasculitis patients and to become an effective vasculitis investigator, preferably in Ireland. The potential exists for participation in international vasculitis collaborative research networks, which could benefit vasculitis patients in Ireland and elsewhere.

Otolaryngologist Performs Reconstructive Surgery for WG Patients - Robert S. Lebovics, M.D., F.A.C.S.

Robert S. Lebovics, M.D., F.A.C.S. is a nationally known Otolaryngologist with expertise in infectious and inflammatory disorders for both adults and children, nasal and sinus surgery, and disorders of the special senses. Dr. Lebovics is a New York native who received his medical degree from the State University of New York in Brooklyn and trained at the Albert Einstein/Montefiore Hospital Center in otolaryngology. For nearly nine years, Dr. Lebovics was Chief of the Clinical Otolaryngology Service at the National Institutes of Health in Bethesda, Maryland.

In 1997, Dr. Lebovics joined the Head and Neck Surgical Group, affiliated with the St. Lukes-Roosevelt Hospital Center. In 1999, Dr. Lebovics was appointed Chief of Otolaryngology/Head and Neck Surgery at Cabrini Medical Center in New York City.

Among his accomplishments, Dr. Lebovics has authored over 25 articles and text book chapters in the field of otolaryngology head and neck surgery. Dr. Lebovics continues as a surgical consultant to the National Institutes of Health in Bethesda, MD for the Department of Health and Human Services.

Dr. Lebovics has seen approximately 200 WG patients including patients from Central America, Mexico, Canada and Ireland. He receives calls from physicians in Australia and Europe seeking advice and tapping into his expertise.

Most of the surgery that Dr. Lebovics performs is in the reconstructive realm. He has performed many surgeries on WG patients in need of reconstruction for saddlenose deformity. His goal for this type of surgery is a fine balance between cosmetic and reconstructive procedures for the best possible outcome.

Dr. Lebovics also performs tracheal repairs on WG patients, a serious operation with no room for mistakes. Few situations in medicine are as black and white as the need for an open airway. Endoscopic dilation is the preferred method but not always sufficient for the repair. Each repair is an individual condition with its’ own problems and peculiarities.

Dr. Lebovics enjoys the work he is able to accomplish in the operating room. With the belief that we need to live for today and do all we can to make each day brighter, he sees his work as a method to achieve this goal.

Dr. Lebovics has learned a great deal from his WG patients - medically and philosophically. There is no limit to the power of the human spirit and no greater lesson than that of redemption. There is always hope, there is a future, do not give up. Dr. Lebovics emphasizes that he can and wants to help his WG patients with the pain, discomfort and deformity that WG so often brings. He loves his WG patients and says they are a fun loving group - some of the nicest people you could ever meet.

During his time at NIH, Dr. Lebovics worked with Dr. Fauci, Dr. Hoffman and Dr. Langford on WG research. There was a tremendous need for the research at the time along with an interest among these doctors that resulted in a “match made in heaven” team. The combination of brilliant physicians and a multidisciplinary approach to the research resulted in great strides within the knowledge and treatment of WG.

Gail Schultz, cosponsor of the NYC support group, says that Dr. Lebovics has been the primary promoter of the NYC support group. He has graciously offered the conference room of his practice for meetings which otherwise would have no place to gather. He attends every meeting and is always available to answer questions. He is dedicated to his patients and once took a patient aside to provide a consultation after one of the support group meetings.

Dr. Lebovics is not only a wonderful and knowledgeable physician, but a great human being as well, says Gail. He truly cares about his patients and in return, he has the admiration of every WG patient in the tri state area (New York, New Jersey and Connecticut).

*Some material excerpted from www.entsurg.com with Dr. Lebovic's permission.

Search Begins for a Genetic Link to Vasculitis - Sharon A. Chung, M.D.

By Sharon A. Chung, M.D., April 2007

My story begins in Raleigh, NC, where I was born and raised.  My father is a retired a physics professor from North Carolina State University (NC State) and my mother is an artist.  My sister is 5 years older than me and a pediatric ER doctor in Boston. 

I must say that I was extremely lucky growing up—the schools I went to had a strong emphasis on science, and everyone around me was tremendously supportive of my interests.  One of my most influential experiences was attending the North Carolina School of Science and Mathematics (NCSSM) for my last 2 years of high school.  While at NCSSM, I took a class called “Research in Biology”.  Although it had a very simple name, I cannot underestimate the impact of this class on me.  It introduced me to molecular biology, and allowed me to develop an independent research project on yeast RNA viruses.  The results of the project were definitely not earth shattering—but the project itself jump-started my desire to conduct research studying human diseases. 

After high school, I went to NC State and obtained a degree in Chemical Engineering.  I had an inkling at that time that I wanted to go to medical school, and to continue research exploring medical diseases.  However, I knew that I wasn’t a “pure science” person—that is, that I enjoyed applying science towards something more than the just discovering the science itself.  Therefore, I obtained an applied science degree—engineering.  While at NC State, I became interested in genetics, and began taking classes in this area, with the idea that I may do research in this field in the future.

After graduating from NC State, I went to medical school at Johns Hopkins University in Baltimore, MD, and stayed at Hopkins for my residency in internal medicine.  I realized 2 extremely important things while at Hopkins—First, that I enjoyed seeing patients far more than I enjoyed growing yeast or working a pipette in a lab.  Second, I was introduced to rheumatology, and became fascinated with this area of medicine.  I remember the vasculitis clinics I attended with John Stone and David Hellmann.  I saw such a range of diseases during that time—from Wegener’s granulomatosis to Takayasu’s arteritis to Behçet’s to CNS vasculitis.  I remember asking myself—“How do these diseases start?  We believe that the immune system loses the ability to distinguish itself from foreign cells—but why?  Is there anything that makes it more likely to happen—genetics, environmental exposures, infections?  Why does it affect some, and not others?” Given my previous interest in genetics, I realized that I had found my calling—to study the genetics of autoimmune diseases, and to use genetics to learn how these diseases develop.   

I came to University of California, San Francisco (UCSF) for a rheumatology fellowship, in part, because of the well-developed genetics research program in rheumatology at UCSF.  Dr. Lindsey Criswell, my mentor, has significant experience studying the genetic epidemiology of both rheumatoid arthritis and lupus.  With her, I have developed a research project to identify genes related to autoantibody production in lupus.  These autoantibodies are believed to play a role in starting lupus.  By understanding which genes are involved in producing these autoantibodies, we may gain insight into the genetic causes of lupus.

My research goal is to expand this work in lupus genetics to other autoimmune diseases, such as vasculitis.  In the future, I would like to study how genes, and which genes, influence the risk of developing vasculitis.   This knowledge may help us predict who is at risk for disease, develop potential treatments, identify which treatments may be more effective for a particular patient, or perhaps even prevent disease.  These are definitely long-term goals—we are not close to achieving them currently—but the potential for reaching these goals is growing! 

I don’t want to give the impression that the only thing I think about is research—quite the opposite!  My clinical practice at UCSF is an important and valuable aspect of my rheumatology career.  I currently see a wide variety of patients with different autoimmune diseases, including vasculitis.  However, due to my interests, I am interested in seeing more vasculitis patients in my clinic and developing an expertise in this field.  In addition, I am spending a month this year at the Vasculitis Center at the Cleveland Clinic to expand my experience caring for these patients.

I find it extremely rewarding to care for vasculitis patients.  Often times, I first meet them when they are quite ill—for example, wheelchair bound due to nerve damage, or in the intensive care unit (ICU) due to organ failure.  These patients have shown me the body’s amazing capacity to heal.  My patient who was wheelchair bound is now walking on her own; the patient who was in the ICU is now a college student.  It is uplifting to see my patients make such significant strides in their quality of life.  I’d like to think that I have some impact with medications and a caring ear, but I know that my patients have a significant role on their own recovery.  Their positive outlook, dedication to medical care, attention to physical therapy, self-education, determination to improve—all of these aspects have helped them progress and I am proud of their accomplishments. 

This July, I will finish my rheumatology fellowship and join the faculty at UCSF.  I look forward entering a new stage of my career and developing a stronger relationship with the vasculitis community!

Subglottal Stenosis in Wegener's Patients - Robert Lorenz, M.D., F.A.C.S.

Almost all WG patients have, to some extent, involvement with their nose and ears. However, few WG patients have problems with their larynx. Only 20% of adult WG patients have subglottal stenosis. This jumps to 50% in patients under 20 years of age, the reason for this unknown. In children, the SS can remain as an issue for years. Dr. Lorenz finds that as long as the WG is not active, the SS stays quiescent . Once the patient has an exacerbation, the SS can flare and then the body’s natural ability to create a stenosis from the scar occurs and the physician and patient struggle to get the airway widely patent while preventing further scaring. SS is a disease that you either have or you do not - there does not seem to be “a little subglottal stenosis”.

Dr. Lorenz has a basic philosophy when it comes to treating subglottal stenosis. If a person is not limited in their activities by their pathology then there should be no intervention. This is an extremely important point. Some physicians may tell a patient that they have a little subglottal stenosis and that something should be done. Unless a patient says they are having problems, Dr. Lorenz will not perform surgery. The only exceptions to this are in patients who are anticipating problems, such as someone needing to undergo general anesthesia and their airway is too tight to get a breathing tube down, or in a pregnant woman who will need a widely open airway to go through the added stress of labor without a restricted airway, but these scenarios are very rare.

This ties into the second part of his philosophy; the more you intervene, the more scarring that will result along with long-term problems. The larynx is an extremely delicate organ and the more you traumatize it, the less chance of recovery in the future. Dr. Lorenz says “Be gentle and do the least amount of trauma to the voice box“.

An important point to understand about subglottal stenosis is that it acts independently of generalized WG disease and systemic therapies. If somebody flares, it doesn’t mean the larynx will get worse. If the larynx is getting worse, it does not mean you are in a WG flare. Lastly, it means that systemic immunosuppression usually does not help reduce the severity of the subglottal stenosis.

Problems do not usually arise unless there is a 50% or greater reduction in the airway. At this point, one returns to the philosophy of “are you having problems?”. You may have a 50% reduction yet not have any significant problems and so you would not have any treatment. Older patients seem to be more subjectively affected with SS - patients over 50 tend to feel more confined by it while younger patients tend to do better.

Dr. Lorenz tries to perform as few surgeries as possible and when he does, the goal is to be as atraumatic as possible. Under general anesthesia, working through the mouth with no tracheotomy or neck incisions in a "minimally invasive", endoscopic manner, the first part of the treatment is the injection of localized steroids which seems to be the most important part of preventing the stenosis from returning. Next, the stenosis is lysed with a small endoscopic knife, and thereafter, the stenosis is lightly dilated. It is very important that the dilation part of the procedure is done very gently so that no further scar tissue is caused. Lastly, a topical application of mitomycin-C is used and the patient is awakened. So far, as long as the patient has not had previous airway surgery by a different surgeon prior to Dr. Lorenz, he has never had to perform a tracheotomy or other more invasive surgeries, and the minimally invasive approach has been able to maintain the airway open. Repeat procedures may be needed at intervals of 8-12 months, but usually are limited to 3 procedures.

Dr. Lorenz does not personally perform the radial incision in the larynx with the laser because there is some evidence that lasers are more traumatic to the larynx in animal studies, but the new generation of lasers may be able to avoid this problem.

Most ENT’s are surgeons, but are generalists who treat all things such as allergy, children, adult, cosmetics, ear, sinus, etc. There are very few with significant interest and experience with Wegener’s and other vasculitides. There are only a handful of ENT’s who have significant experience with subglottal stenosis.

Disease in the sinuses ranges from minor inflammation all the way to tissue destruction. At the least, there is usually some redness, swelling and some crusting. In the worst cases, the septum has completely eroded leaving a large nasal cavity with no real sinuses left.

The difference between disease in the nasal cavity and in the sinuses is as follows … Disease in the nasal cavity centers around crusting, bleeding and a destroyed septum. Disease in the air-filled spaces of the sinus cavity is primarily infection or sinusitis. Sinus surgery is rarely performed when a person is in a WG flare. Once a diagnosis comes back, organ systems are checked, treatment with meds is decided to bring the WG under control and with proper treatment, the nose tends to get better and surgery can be avoided.

It is extremely important to take good care of your nasal and sinus passages. Proper hygiene is the primary method of preventing problems in our WG patient population of approximately 200. It is important for all patients to know the complications that can arise from poor hygiene. They include acute sinusitis, mucoceles (walled off area of mucous which can eventually involve neighboring tissues like the eyes and brain), and ear problems.

Dr. Lorenz performs surgery only with the larynx in WG patients. If care is needed for the sinuses, he refers them to Dr. Citardi or Dr. Batra, both also at the CCF. Ear problems are referred to a specialist as well.

Dr. Lorenz is from New York, north of New York City in the Hudson Valley. His parents were immigrants from Europe after WWII. He currently lives with his wife, who is also a physician, in Shaker Heights, Ohio.

When Dr. Lorenz is not in Cleveland seeing patients and performing surgery, he spends much of his time lecturing. In May he was in Chicago followed by trips to Colombia, Panama, Turkey and then on to Baltimore for the Vasculitis Foundation Symposium in July. When he is not taking care of patients, he likes to relax at home with his wife, Aylin.

As a college student at Yale, Dr. Lorenz became involved with an international medical equipment aid group and visited hospitals in Central America. This is what really inspired him to become a doctor. He is the first member of his family to become a doctor. Dr. Lorenz has also been on medical mission trips to Africa.

Dr. Lorenz credits Drs. Isaac Eliachar and Gary Hoffman as fantastic teachers who taught him an incredible amount about vasculitis patients. Dr. Bob Lebovics and Dr. Carol Langford have also been excellent collaborators.

Dr. Lorenz states that the best part of his job is making a person who has a severe airway narrowing which affects their daily life, breathe normally again. He feels that WG patients are wonderful people - folks just like him, but were very unlucky to develop a disease that could affect anyone. His practice is limited to laryngotracheal reconstruction and Head and Neck tumors.

Dr. Robert Lorenz is an ENT physician and surgeon at the Cleveland Clinic Foundation (CCF) in the Department of Otolaryngology, where he is Section Head of Head and Neck surgery. His area of special interest for Wegener’s patients is the larynx. WG is unique in its ability to involve the larynx while the other vasculitides do not. When subglottal stenosis affects the larynx, the cause can be either WG or idiopathic (of unknown origin), or sometimes due to trauma such as breathing tubes.

Vascular Surgery in Patients with Vasculitis - Ravi Veeraswamy, M.D.

By Ravi Veeraswamy, M.D.[img_assist|nid=1481|title=Dr. Ravi Veeraswamy

As a vascular surgeon, I am frequently asked to perform temporal artery biopsies to make a diagnosis of arteritis.   This is perhaps the most common referral I receive.

Other physicians not familiar with the disease process also often refer vasculitis patients to vascular surgeons.  The physician and the patient are concerned about arterial insufficiency or arteriosclerosis as the cause.  For example, I recently referred a gentleman in excellent health without any risk factors for arteriosclerosis who noted that his fingers became discolored on several occasions in cold temperatures.  The role of the vascular surgeon is to identify the underlying process and refer the patient to an appropriate specialist, usually a rheumatologist.  The most common type of referral in this category is for patients with Raynauds Phenomenon.  Often, the presentation can be confusing and we will perform a diagnostic angiogram to help define the problem and rule out other causes such as proximal aneurysms or other embolic sources.

The other end of the spectrum is when patients are referred to us for “end-stage” disease.  Patients have developed chronic stenosis of vascular beds such as the upper extremities or the carotid circulation and they need bypasses to restore arterial flow.  In this scenario, it is the rheumatologist who sends the patient to us.  Patients typically need bypass grafts placed distal to the areas involved in the inflammation.  For example they may need bypasses from the aorta to the carotid or from the carotid to the brachial and so on.   The decisions on specific bypasses are based on a given individual’s arteriograms and anatomy.

We typically are “repairing damage” rather than preventing it.  There are really no great surgical treatments currently to help prevent damage in the setting of vasculitis.  I first encountered patients with vasculitis in the surgical clinic as a medical student.  I was amazed at how little we knew about the root causes of the problem.  While we have learned a lot since then, there is a long way to go.  My research interest has always been in immunobiology and in managing patients with vasculitis, I am able to combine my clinical and research interests.  Currently, I am establishing research projects to study the role of dendritic cells and T cells in vascular biology and the arterial wall.  Hopefully this will help us understand the process that initiates the inflammatory response and give us insight into how it might be controlled.

On a personal note, I have wanted to be a doctor literally for as long as I can remember.  I used to dress up as a doctor all the time as a kid.  I still find the profession fascinating and consider myself very lucky to be in a field where I can directly help other people.  I think the best relationship is when the doctor and the patient work together with a common understanding of the possibilities of treatment and the limitations of treatment.  The best thing a vasculitis patient can do is find a good rheumatologist with an interest and experience in managing the vasculitis.   

Vasculitis Center to Open in Salt Lake City, Utah - Curry L. Koening, M.D.

Carol A. Langford, M.D., MHS

Director, Center for Vasculitis Care and ResearchDepartment of Rheumatic and Immunologic Diseases

Cleveland Clinic Foundation

 As Dr. Koening discusses in his interview, he is one of a group of talented young investigators who have been selected to receive training through the Vasculitis Clinical Research Consortium (VCRC) that is sponsored by the National Institutes of Health. The establishment of a vasculitis fellowship through the VCRC was vigorously pursued and supported by the VCRC’s Principal Investigator Dr. Peter Merkel and the entire VCRC steering committee. The education of physicians to become vasculitis investigators represents one of the most important contributions that we may bring to the care of people with vasculitis and to the future of our field.

Dr. Koening and all of the VCRC scholars exemplify the promise that this future holds. Upon completing his vasculitis fellowship, Dr. Koening will be taking the skills that he has gained back to the University of Utah where he will be establishing a Vasculitis Center. This Center will serve as a vital resource to people with vasculitis in the Western United States and as a valuable partner in ongoing research studies. It has been a great pleasure for all us in Cleveland to work with Dr. Koening and we look forward to the opportunity to collaborate with him for many years to come.

By Curry L. Koening M.D., February 2006 

I first realized that I wanted to be doctor when I was in high school. I grew up in a small town in West Texas. Farming and ranching were how most families made a living in this part of the country. Oil also played a huge part in the local economies. However, lack of primary medical care was a major issue in many of these small towns. Not many doctors were moving to these rural areas to start a practice, leaving many with limited access to medical care. I began medical school with the intent of moving back to rural Texas and starting a general medical practice.

After college I went to medical school at Texas A&M System Health Science Center College of Medicine. I was first introduced to rheumatology during my third year clerkship. I decided to pursue it as a profession after spending some time on the rotation during my internal medicine residency at the University of Utah. Rheumatology was not unlike primary care. It too needed new, young doctors to help underserved areas, especially where wait times to see a rheumatologist may have been as long as six months. I completed my rheumatology fellowship at the University of Utah in June of 2005 and moved to Cleveland shortly thereafter to learn more about vasculitis at the Center for Vasculitis Care and Research at the Cleveland Clinic Foundation. Because few doctors have trained specifically in vasculitis, patients with these diseases are underserved. Research in this field is also lacking and clinical trials for new therapeutics have lagged behind.

I am fortunate to be receiving support for my vasculitis training from funds provided by the National Institutes of Health through the Vasculitis Clinical Research Consortium (VCRC). It is the mission of the VCRC to train physicians to serve patients with vasculitis and through research provide better therapies, diagnostic tools and identify the causes of these diseases. I am fortunate to be a part of this organization and I look forward to playing a greater role in the care of patients with vasculitis and leading research studies in the future.

The Vasculitis Clinical Research Consortium (VCRC) has four main clinical centers: Boston University School of Medicine, Cleveland Clinic Foundation, Johns Hopkins School of Medicine and Mayo Clinic College of Medicine. The goal of the VCRC is to conduct clinical research in hopes of developing new treatments for patients afflicted with vasculitis. It has been the goal of the VCRC to train new clinicians to care for patients with vasculitis, conduct clinical trials for new drugs in the treatment of vasculitis, and work with vasculitis patient support groups. My goal as a trainee through the VCRC is to maintain this tradition. My training will consist of two years of research, patient care and academic work. This includes not only learning how to better care for patients with vasculitis, but also graduate work in how to perform basic clinical research that will serve to ultimately improve our patient’s daily lives.

My current research interest is in disease monitoring. Little is known regarding how best to follow patients with vasculitis who are considered to be in “remission”. For most patients who are on immunosuppressant drugs, regular blood and urine monitoring are a requirement. How to follow patients who are in a low disease state or even disease remission and not on immunosuppressant drugs is much less clear. Do these patients need regular blood monitoring to detect an early disease relapse? Do these patients need regular CAT scans or chest x-rays to detect an asymptomatic lung relapse of their disease? If so, then how often do they need to be seen and is it cost effective to do regular lab monitoring? These are questions that still remain and that future research will address.

My first encounters with WG and other forms of vasculitis were during my residency. As you might guess, these experiences were infrequent. Nonetheless, I had the opportunity to care for a few patients with WG, Takayasu's arteritis, Churg-Strauss and other forms of vasculitis when they were hospitalized. I learned in my fellowship that patients with vasculitis often have life-threatening complications, but with prompt and proper therapy they can do very well.

The number of vasculitis patients that we see at the Cleveland Clinic varies from week to week.   About 50 patients with vasculitis will be seen on average per week. WG patients make up a large number of those seen and is one of the most common vasculitides that we take care of at the Center for Vasculitis Care and Research. Other vasculitides that we see commonly include Takayasu’s arteritis, giant cell arteritis, polymyalgia rheumatica, polyarteritis nodosa, Churg Strauss and Behçet’s. The Center for Vasculitis Care and Research has one of the largest populations of WG patients worldwide. Not only has the Center committed itself to the care of patients with vasculitis but also to increasing our knowledge about these diseases through clinical trials and basic research.

As a fellow in vasculitis my job entails many things:

1) Designing and participating in research.

2) Participating as a co investigator in clinical trials.

3) Seeing and treating new and established patients who have vasculitis.

4) Working closely with clinical staff members who have cared for vasculitis patients for many years and learning from their experiences.

5) Being available to other clinicians world wide to answer questions and to help better serve patients with Vasculitis.

6). Teaching medical students and residents basic approaches to diagnosing and treating Vasculitis.

7). Completing academic work in a pursuit for a master’s degree in the Clinical Research Scholars Program at Case Western Reserve University.

The importance of communication has been the most helpful thing that I have learned during my training. I have found that taking the time to discuss any concerns that patients have and helping them develop a plan to deal with their disease has greatly enhanced my relationship with my patients. In this day and age of an electronic medical record and e-mail there is no reason that patients should ever feel “left out of the loop.”

I think there are some things that we as physicians and patients can do to make things easier for both. First, I think it is very important for patients to have a vested interest in their disease. In this way, patients will be more apt to report changes that they may recognize as a disease flare. Patients will also be prompted to ask questions that are important to their own care. Patients should be optimistic about the progress that is being made that has led to better care for patients with vasculitis. Progress in the field of vasculitis research can not happen without the help of patients. I think it is important for patients to keep an open mind when it comes to participating in clinical trials. The more people that are willing to participate in trials, the better our understanding of these rare diseases will be. Patients should also be involved in their local Vasculitis support groups. It is through these groups where patients can learn about new therapies and how they can support future research in this area.

I think the best advice for any vasculitis patient is to recognize that recent medical advances have made not only long term survival but long term disease remission a reality. Even though disease remission is a reality for almost all patients, a cure is still many years away. Patients should continue to have frequent follow-up visits with their care providers, ask pertinent questions and be involved with their local and national Vasculitis organizations. This also includes patients who have been in disease remission for an extended period of time. These patients also have a high risk of relapse from their disease. Vigilance can prevent complications. Early detection of relapses can lead to early treatment, shorter exposure to immunosuppressant drugs, less morbidity and hopefully a faster return to normal daily living.

I have several goals that I would like to accomplish before my time is complete at the Cleveland Clinic. My ultimate goal is to learn how to design and implement clinical trails for the treatment of patients with Vasculitis. I would also like to be a part in developing new imaging and laboratory modalities that will help how we follow patients in the future. Through my course work at Case Western Reserve University and clinical time at the Cleveland Clinic, I hope also to forge collaborations worldwide with clinicians and researchers who are dedicated to better understanding Vasculitis and the patients we care for. Finally, as any young physician I want to provide the most advanced therapies for my patients in a compassionate clinical setting for which patients can feel a part of.

My plans at this time are to take an academic appointment at the University of Utah in Salt Lake City. Here, in conjunction with the Department of Medicine and Division of Rheumatology, we plan to create a vasculitis center. This will be the first Center in the Western United States. Our goal is to be a tertiary referral center for patients from all over the United States and the World who are afflicted with either WG or other rare vasculitides. We will also offer clinical trials of new therapeutics to treat patients with vasculitis that would not otherwise be able to participate. We also hope to build a strong relationship with local and national Vasculitis support groups such as the Vasculitis Foundation and maintain a strong collaborative network with the Centers for Vasculitis Care and Research and other Vasculitis centers around the world.

Young Investigator Seeks Answers Through Epidemiological Studies - Alfred D. Mahr, M.D.

Peter A. Merkel, M.D., M.P.H.

Principal Investigator, Vasculitis Clinical Research Consortium  

Director, Boston University Vasculitis Center

Associate Professor of Medicine

Boston University School of Medicine

It is a great pleasure to introduce Dr. Alfred Mahr to members and friends of the Vasculitis Foundation.  Dr. Mahr has been a Research Fellow and Visiting Scholar at the Boston University Vasculitis Center since November 2005.  He is one of four current Fellows supported by the Vasculitis Clinical Research Consortium (VCRC) which receives its funding from the National Institutes of Health.  One of the core missions of the VCRC is to train new investigators in vasculitis and thus help expand the number of caregivers and researchers in vasculitis. It is hoped some of the Fellows will go on to establish new centers of excellence for vasculitis. 

Unlike the other current VCRC Fellows, Dr. Mahr was already an investigator in vasculitis when he joined us here in Boston.  As a member of the French Vasculitis Study Group in Paris, Dr. Mahr studied under Dr. Loïc Guillevin.  Dr. Mahr was a productive researcher in vasculitis and a clinical expert in the treatment of these difficult diseases.  We were thrilled that Dr. Mahr wished to come and study here at Boston University where he is pursuing advanced courses in clinical epidemiology and biostatistics, and has designed and is implementing novel research studies.  The research projects he has developed includes clinical trials, clinical epidemiology, and genetic studies; the diseases his research covers includes Wegener’s granulomatosis, microscopic polyangiitis, Churg Strauss vasculitis, and giant cell arteritis.  His first year as a VCRC Fellow has been a productive one and his second year will be even more exciting.

The support of clinical and research fellows in vasculitis is instrumental to not only continuing to provide care and conduct research, but also to expand the scope of care and research in vasculitis.  The continued support from the NIH and the Vasculitis Foundation makes this training possible.  The impact that young investigators have on the productivity and conduct of research should not be underestimated.   

Young Investigator Seeks Answers Through Epidemiological Studies

By Kathy Savickas, December 2006

Dr. Alfred Mahr was born in Germany and at the age of four he moved to France for the next five years.  His whole family fell in love with France and though they returned to Germany when he was nine, they visited often for vacations.  France would become Dr. Mahr’s second home.

Dr. Mahr knew in high school that he wanted to attend medical school.  His father was an engineer and his mother a physical therapist but his grandmother, great-grandfather and great-great-grandfather were also doctors.  Dr. Mahr knew that he wanted to work with people and medicine was a perfect balance between an intellectual challenge and humanitarian work.  That mixture drove him to become a doctor and is what continues to motivate him today.

Once Dr. Mahr finished high school in Germany, he moved to Montpellier in the south of France to attend medical school, the oldest in the country, and complete his residency in internal medicine. He then moved to Paris to specialize and complete his fellowship.  Dr. Mahr’s wife, Laurence, is also a physician and they have a five-month-old baby named Simon.

While doing rounds in various specialties, he met his first vasculitis patient in 1995 when he was a fellow in internal medicine.  The patient initially presented with fevers and muscle aches.  It took quite a while to unravel the nature of the patient’s disease before he could be diagnosed with Polyarteritis nodosa. Soon thereafter, he saw an elderly lady with Microscopic polyangiitis involving the kidney. At that time, Dr. Mahr became intrigued by vasculitis.  He felt that, because they were rare and presenting so differently from patient to patient, vasculitides were more difficult to understand and recognize than many other diseases he has taken care of since then.

In 1997 he decided to complete rounds with Dr. Loïc Guillevin who is the head of the Department of Internal Medicine at Hôpital Cochin in Paris and a prominent expert in the field of vasculitis in Europe.  Folks come from many countries to seek his help.  During his rounds with Dr. Guillevin, Dr. Mahr came to see many patients with different forms of vasculitis. At that time, he decided to devote his medical thesis to Wegener’s granulomatosis. After completion of his fellowship in 1998 he accepted an offer to return to Dr. Guillevin’s department as a faculty member, a position that he has not left until now. Since 1998, he has continuously worked in the field of vasculitis and has learned to take care of various types of vasculitis including Wegener’s granulomatosis, Microscopic polyangiitis, Polyarteritis nodosa, Behçet’s disease, Churg Strauss syndrome, giant-cell arteritis, Takayasu’s arteritis, and others.

During this time, Dr. Mahr also completed a Master’s in Public Health and began to foster his personal research by combining his interest in vasculitis with epidemiology.  Epidemiology can be defined as a field of study that searches to understand the triggers and causes of diseases.  Dr. Mahr has accomplished different studies conducted on the prevalence of vasculitis in the Parisian region, which include an investigation of factors that predict the severity of vasculitis and a study of links between use of particular anti-asthma drugs and Churg Strauss Syndrome. Dr. Mahr is also working on his Ph.D. thesis in Public Health, which he plans to submit in 2007 on the theme of vasculitis.

While attending various meetings, Dr. Mahr met Dr. Peter Merkel who, in time, invited him to come to Boston and work on vasculitis research by enrolling in a fellowship program with the Vasculitis Clinical Research Consortium (VCRC).  Boston proved to be an excellent location for Dr. Mahr because the Vasculitis Center in Boston has both the skills in vasculitis and the tradition of doing epidemiological studies.  The position couldn’t be more perfect for the studies that he is currently working on.  He took a sabbatical from his position in Paris for two years and came to Boston in November 2005.

Dr. Mahr’s primary goal as he works in Boston is to become more skilled in research.  He is grateful he has the opportunity to study here and is learning a lot in the United States from a scientific and a personal standpoint. He is taking advanced classes in statistics, genetics, and research methods.  Dr. Mahr is also conducting new studies of Wegener's granulomatosis, Churg Strauss, giant cell arteritis, and other topics.  When his training in Boston is completed in November 2007, Dr. Mahr plans to return to Paris where he will continue to work with Dr. Guillevin. However, he hopes to continue collaborating with physicians from the United States as well as from other parts of the world. For rare diseases such as vasculitis, it is crucial that physicians and researchers from different countries combine their efforts to keep moving forward in the understanding and treatment of these illnesses.

Dr. Mahr enjoys taking care of his patients.  He feels privileged in his role as their doctor and of being able to assist them while they are going through a difficult patch. He feels that a good relationship between a patient and his doctor should be built on one of complete trust and confidence.  Dr. Mahr encourages his patients to tell or ask him everything they have on their mind. His motto is “There is nothing to hide” and “There are no stupid questions”!