We do not know that there is any increased risk for vasculitis in your children. Increased risk for family members would suggest that there is either an inherited factor that is responsible for developing vasculitis, or that there is a common exposure that causes disease. To date, there is nothing to suggest that either of these factors causes vasculitis. Testing to try to identify vasculitis in anyone, whether a family member of a patient or not, should be driven by specific symptoms or medical problems.
The first step to find a specialist with expertise in vasculitis treatment is to ask your primary physician. Many patients with vasculitis are cared for by rheumatologists. The American College of Rheumatology has a tool on its website to help locate a rheumatologist by geographic site and many local chapters of the Arthritis Foundation maintain lists of local rheumatologists. For physicians with expertise in vasculitis care, the Vasculitis Foundation can also offer suggestions. Some patients with vasculitis affecting specific organs need care from other specialists, such as pulmonologists (lung disease specialists) or nephrologists (kidney specialists).
This is a complicated question and raises issues about trust and confidence in one’s physician. Because of the complexities in diagnosing and treating vasculitis, patients with vasculitis should receive care directed by a physician with expertise in vasculitis. Seeking a second opinion might be considered if there is a problem making a diagnosis, or to help answer questions about therapy, especially if current therapy is not effective or complicated by adverse reactions.
There is no simple answer. Specific data are not available and it is hard to answer as there are many different types of vasculitis, some of which are mild and others which are more severe. Some forms of vasculitis can affect vital organs and be life-threatening when the disease is active. Vasculitis can also cause damage to organs that can affect overall life expectancy. In addition, use of medications to treat vasculitis that suppress the body’s immune system can increase the risk of infection. It is best to address this with your own physician to take into account your particular type of vasculitis and problems that might have arisen from the disease and its treatment.
This question is difficult to address in general terms because there are a lot of individual factors that influence the need for specific laboratory tests. In principle, serial laboratory testing over time has two major goals. First, laboratory testing should screen for disease activity. Second, laboratory testing should look for toxicity of the medications used to control the disease activity. The following battery of tests is usually sufficient for most types of vasculitis; markers of inflammations (erythrocyte sedimentation rate and C-reactive protein), a complete blood count (with differential cell count), a blood chemistry panel (that includes liver function tests and kidney function tests), and a urinalysis with urine microscopy (for forms of vasculitis that frequently involve the kidneys). The frequency of these tests depends on the individual situation (type of disease, organ system involvement, medications used, whether remission has been achieved, and the duration of remission, etc.) In addition to laboratory testing, your doctor may also choose certain imaging studies such as chest x-ray, chest CT (CAT) scan, MR angiograms (MRI), or others, to follow specific disease manifestations in specific forms of vasculitis.
Disease activity represents the aspects of your disease that are caused by active inflammation in organs targeted by the vasculitis and response to treatment with steroids and immunosuppressive drugs. Disease damage represents the parts of your disease that do not respond to anti-inflammatory and immunosuppressive therapy. In any particular patient, damage might include problems caused directly by your disease (such as chronic kidney problems or neuropathy causing numbness in the feet), or problems caused by the drugs used to treat vasculitis (such as diabetes or high blood pressure, both of which can be caused by steroid use).
Severity can have many definitions. From an individual patient’s perspective, severity can be viewed impact the vasculitis has on their overall functioning or the functioning of an individual organ system. From the perspective of your treating physician or a researcher involved in vasculitis, severe disease usually is taken to mean involvement with potentially life-threatening or organ-threatening disease. Involvement of the kidneys, brain, eyes, heart and intestines, as well as bleeding into the lung, is typically considered features of “severe” disease.
The earlier that vasculitis is identified and treated the greater the chance of recovery from nerve damage. Unfortunately, many times recovery may only mean a partial recovery that may take many months or years to happen.
Yes, other health issues have to be kept in mind in patients who have vasculitis. Some can be related to vasculitis directly and others can be related to side effects of treatment of vasculitis. Those related to vasculitis directly include things such as stroke, heart attacks, nerve damage causing weakness, lung damage causing shortness of breath and cough and kidney damage that may lead to swelling of the legs and dialysis. Issues related to treatment include such things as osteoporosis, high blood pressure, high cholesterol, infections and development of certain types of cancers.
This is a general question that cannot be answered specifically without knowing the patient’s individual situation. A well-balanced diet following recommendations of the American Heart Association is usually sufficient. In patients with high blood pressure, a low-salt diet maybe necessary. Patients with renal insufficiency will have specific restrictions of protein and potassium intake. Specific dietary recommendations also apply to patients with diabetes, which in turn may be worsened by the use of glucocorticoids. A consultation with a dietitian who can factor in an individual patient’s specific needs is certainly a good option for some patients.
Yes, patients with vasculitis should get a flu shot every fall. The treatments taken by most patients with vasculitis make them at higher risk of getting infections like influenza A, which could be severe. Patients should get the regular flu shot, which is a “killed virus” vaccine an unable to cause infection, rather than the nasal spray version, which is a “live attenuated” virus and able to cause infection in people who receive it, a special problem for patients taking immune suppressing treatments. The University of North Carolina – Chapel Hill Kidney Center developed an extensive podcast on immunizations. Click here to listen.
Prednisone directly causes atrophy of muscle fibers (myopathy) leading to muscle weakness. This is a very common side effect of chronic (a few months or more) prednisone use but can be either mild or severe. Patients usually notice weakness most in the upper legs. It is not a painful condition directly, but weak muscles and their tendons are more easily strained, which can lead to pain. Muscle weakness that is so severe as to involve the muscles of breathing is rare, but leg weakness can lead to shortness of breath with climbing stairs.
Not exactly. Even though asthma is present in almost all patients with Churg Strauss syndrome and corticosteroids represent the cornerstone treatment for both conditions, asthma it is not part of the vasculitis. In Churg Strauss syndrome, asthma is more to be considered as an underlying predisposing condition, and it usually persists even after effective treatment for vasculitis.
“Poly” indicates that many blood vessels are involved, and “angiitis” is used instead of “arteritis” because granulomatosis with polyangiitis (Wegener’s) and microscopic polyangiitis affect the smallest vessels in the body (arterioles, capillaries, and venules), all of which are smaller than arteries. In contrast, in polyarteritis nodosa, giant cell arteritis, and Takayasu’s arteritis, only arteries (either a little or much larger than arterioles) are affected.
Both are blood tests used by doctors to help in the diagnosis of autoimmune disease. Antineutrophil cytoplasmic antibody (ANCA) is a blood test commonly elevated in patients with diseases such granulomatosis with polyangiitis, microscopic polyangiitis, and Churg-Strauss syndrome. Antinuclear antibody (ANA) is a blood test most often elevated in patients with systemic lupus erythematosus (“lupus”), Sjogren’s syndrome, scleroderma, and other types of autoimmune diseases.
Hearing can be transiently decreased in patients with GPA with acute otitis, because of the presence of liquid in the inner ear(s), which will regress under appropriate treatment. However, when the inner ear damage is severe, because of prolonged or multiple recurrences of otitis, and/or when the auditory nerve(s) are involved by inflammation or compression, the hearing loss can be permanent.
Studies have been performed that suggest that rituximab works well in cryoglobulinemic vasculitis particularly when associated with hepatitis C infection, and some of these studies are already published. Other types of vasculitis in which rituximab seems likely to work (polyarteritis nodosa, Churg-Strauss) are more difficult to study due to their rarity. It seems unlikely that rituximab would be effective in giant cell arteritis or Takayasu’s arteritis, and therefore it may be a long time before studies are done in these diseases.
Anti-GBM antibodies are antibodies directed against glomerular basement membranes. Patients with anti-GBM disease (Goodpasture’s syndrome) will present with involvement of lungs and kidneys, so-called pulmonary-renal syndrome. This can include bleeding in the lungs (hemoptysis) that can lead to respiratory failure and bleeding in the kidney (hematuria) that can lead to rapidly progressive loss of kidney function. Goodpasture’s syndrome has to be considered in patients who are thought to have ANCA-related vasculitides (granulomatosis with polyangiitis and microscopic polyangiitis) as those diseases can also present with pulmonary-renal syndrome. Measuring anti-GBM antibodies and ANCA in these patients will usually help distinguish the two conditions although there are rare cases of patients with both ANCA and anti-GBM antibodies.