By Jeff Hersh, PhD, MD, FAAP, FACP, FAAEP

One of the nurses I work with has just started volunteering for the Vasculitis Foundation, and she asked me to write a column about vasculitis to increase awareness of these often misunderstood diseases.

In medicine, ‘itis’ is a suffix that means inflammation; for example appendicitis is inflammation of the appendix.  Vasculitis is inflammation of the vasculature (blood vessels), including the arteries and/or the veins.  Inflammation of the blood vessels is characterized by white blood cells (leukocytes) infiltrating the vessel walls.  Although there are many theories as to why this happens, the exact cause is not known for sure.  Some vasculitities are thought to be an allergic-type reaction, and others are thought to be autoimmune (the body attacking itself with its own immune system).  This inflammation and the body’s subsequent reactions to it can cause the vessels to become weakened and can impair the integrity of the vessels walls, which in turn can prevent adequate flow of blood through the affected vessels.  This can cause ischemia of the organ and body tissues fed by this blood supply, and this is responsible for many of the problems that the different types of vasculitis can cause.

Although my goal today is to discuss vasculitis in general, it should be noted that there are many different types of vasculitis.  Vasculitis can be a complication of some other disease (and hence would be called secondary vasculitis), such as infections, malignancy, reactions to certain medications, as a complication after organ transplant, from a connective tissue disease or from other causes.  Vasculitis can also be a primary disease, that is not associated with another cause.   The different types of vasculitis are usually first categorized by what size blood vessels are involved.  Examples of large vessel vasculitis are temporal arteritis (also called giant cell arteritis) and Takayasu arteritis.  Medium sized vessels are involved in polyarteritis nodosa and Kawasaki disease.  Finally, diseases such as Wegner’s granulomatosis, Churg-Strauss syndrome, Henoch-Schonlein purpura and many others are small vessel vasculitities. 

In general, most types of primary vasculitis are pretty uncommon.  For example, Wegner’s affects one to five people per 100,000.  Henoch-Schonlein affects up to 10 per 100,000 and temporal arteritis around 50 per 100,000.  Some diseases affect certain populations more than others.  Temporal arteritis is usually a disease of older people, while Henoch-Schonlein purpura usually affects kids with a peak between ages 5 to 15 years old.  Some ethnic groups have much higher incidences of certain diseases; for example Kawasaki’s disease affects up to 1 in 1000 kids in Japan, although it can affect adults as well.

The symptoms from vasculitis stem from the general inflammatory process as well as from complications of the ischemia to the specific organ system affected.  There are therefore usually some non-specific systemic complaints such as fever, weakness or body aches, as well as specific symptoms depending on the end organ system compromised.  Henoch-Schonlein patients typically present with the triad of abdominal pain, palpable purpura and periarticular (around a joint) inflammation/swelling.  Wegner’s disease can affect the lungs and the kidneys, so patients may complain of symptoms related to these organ systems such as breathing problems from the lung involvement and other problems if their kidneys fail.

The fact that there are many different kinds of vasculitis that affect different organs and cause different symptoms in different patients makes the diagnosis of vasculitis somewhat difficult.  Vasculitis is usually suspected in patients with some systemic symptoms as well as a specific organ involved (such as the skin, lungs, kidneys, etc.).  Screening blood tests to check the function of internal organs (such as blood tests to check for kidney function) are needed.  Other blood tests, such as a test for ANCA

(anti-neutrophil cytoplasmic antibodies) are abnormal in certain types of vasculitis, and can aid in their diagnosis.  However, a biopsy of the most affected organ is usually needed to see the vessel inflammation and to verify the diagnosis.

Despite the difficulty making a definitive diagnosis of vasculitis, early diagnosis is important as these diseases do respond to appropriate treatments.  For example, untreated Wegener’s disease has a 100% mortality with an average survival of only 5 months.  However, treatment of this disease achieves remission in 80-90% of patients, and with treatment the 5-year survival is around 75%.  This illustrates the importance of being persistent to get a definitive diagnosis in patients with possible vasculitis.

There is a classification system by the American College of Rheumatology to help specify which type of vasculitis a patient has, and this is useful in determining which treatments may be best and in helping give the patient an idea of their prognosis.  However, in general this classification system is not really helpful in making the diagnosis of vasculitis, and a biopsy of the most involved organ is considered necessary for a definitive diagnosis.

Since the vasculitities are inflammatory diseases, it is not surprising that the treatments are often steroid-type medications to combat the inflammation, as well as other immune system modifying medications.  Long-term use of these medications is typically required for many types of vasculitities, but the use of these medications is not without a downside.  People who die from vasculitis usually succumb to the disease and its direct complications (such as failure of the affected organ) if they die early in the course of the disease (while it is acutely active), but to complications from the immune suppression and other complications of the medications used to treat the disease when they die later in the course of the disease.  Since stopping the medications can have the disease acutely flare up with the problems it causes worsening, the balance between the complications from the disease and the potential complications from the treatments can be difficult.

If you have vasculitis, a specialist for that disease should follow you.  Effective treatments exist for many types of vasculitis.  Although some types of vasculitis will resolve, some will require on-going long-term treatments, with periodic adjustments of medication to achieve the best balance between disease control and medication complications.  For more information on vasculitis, go to www.vasculitisfoundation.org or see your doctor.

List of diseases

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