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This is a selected article from InFocus, the quarterly newsletter of the American Autoimmune Related Diseases Association. You may obtain full issues of the newsletter by selectig "subscribe," above.
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Vasculitis: A Family of Autoimmune Diseases

     Vasculitis is an inflammation of the blood vessels in the body.  In vasculitis, the body's immune system mistakenly attacks the body's own blood vessels, causing them to become inflamed.  Inflammation can damage the blood vessels and lead to a number of serious complications.

     Vasculitis can affect any of the body's blood vessels.  These include arteries, veins, and capillaries.  Arteries are vessels that carry blood from the heart to the body's organs, veins are the vessels that carry blood back to the heart, and capillaries are the tiny blood vessels that connect the small arteries and veins.  Types of vasculitis include the following:

     Behçet's disease - Behçet's disease can affect any part of the body because it can occur in vessels of any size or type.  Commonly affected organs include the eyes, mouth, and genitals.  Patients with this type of vasculitis are often younger than those with other types, many times in their 20s and 30s.

     Buerger's disease - Buerger's disease typically affects blood flow to the hands and feet.  Buerger's disease is also know as thromboangiitis obliterans and is more likely to occur in smokers than nonsmokers.

     Central nervous system vasculitis (CNS) - CNS's most common symptoms 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.

     Churg Strauss syndrome (CSS) - CSS is a vasculitic disease that involves inflammation of small- to medium-sized blood vessels.  CSS is characterized by asthma and the proliferation of eosinophils, a type of white blood cell.  Damage to sinuses, lungs, heart, skin, gastrointestinal tract, nerves, and occasionally kidneys and joints may occur.

     Cryoglobulinemia - Cryoglobulinemia's most common symptoms are a rash on the lower limbs, arthritis, and nerve damage.  Type 1 cryoglobulinemia is often associated with lymphoma, while Type 2 cryoglobulinemia is often associated with hepatitis C infection.

     Giant cell arteritis (GCA) or temporal arteritis - GCA's common symptoms include headache, fever, blurred vision, and pain in the jaw, shoulders, or hips.  Women, Caucasians, and individuals over 50 years of age are most commonly affected by GCA.  GCA is often called temporal arteritis.

     Henoch-Schönlein purpura (HSP) - HSP most commonly affects the skin, kidneys, joints, and stomach.  It is the most common form of vasculitis found in children but can appear at any age.  When seen in children, it often follows an upper respiratory infection.  It is not usually a chronic disease, and full recovery is common.

     Hypersensitivity vasculitis (HV) - HV is often used to describe different types of vasculitis related to drug reactions, skin disorders, or allergic vasculitis.  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.

     Kawasaki disease - Kawasaki disease is a rare vasculitis which strikes children.  Over 4,000 children develop it each year, and 80 percent of the patients are under the age of five.  Symptoms include fevers; 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 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.

     Microscopic polyangiitis (MPA) - MPA most commonly affects the kidneys, skin, and nerves; but it can affect any organ in the body.

     Polyarteritis nodosa (PAN) - PAN most commonly affects the kidneys, skin, and nerves; but it can affect any organ in the body.  It is most common in people in their 30s and 40s.  Men as twice as likely as women to get PAN.  In some cases, it is associated with chronic hepatitis B infection or a very specific type of leukemia known as hairy cell leukemia.

     Polymyalgia rheumatica (PMR) - PMR most commonly affects the shoulders and hips.  It often occurs along with giant cell arteritis.

     Rheumatoid vasculitis (RV) - RV can affect many different organs, including the eyes, skin, hands, and feet.  It commonly occurs in patients who have severe rheumatoid arthritis.

     Takayasu's arteritis (TA) - TA affects the aorta (the largest blood vessel in the body) and its branches, which carry blood away from the heart.  It typically occurs in Asian women younger than 40 although more and more individuals are being diagnosed with TA.

     Wegener's granulomatosis (WG) - WG can occur anywhere in the body.  It most commonly affects the upper respiratory tract (nose, sinuses, and throat), lungs, and kidneys.  It is most common in people who are middle-aged and older.

Source: Vasculitis Foundation newsletter, November/December 2007, www.vasculitisfoundation.org