Vasculitis means inflammation of the blood vessels, arteries, veins or capillaries. When such inflammation occurs, it causes changes in the walls of blood vessels, such as weakening and narrowing that can progress to the point of blood vessel blockage.
A result of vasculitis is that the tissues and organs supplied by affected blood vessels do not get enough blood. This can cause organ and tissue damage that can even lead to death.
Vasculitis is a family of rare diseases that can affect people of all ages. Though some forms of vasculitis improve on their own, most require treatment. The length of treatment varies, with some people using medications for extended periods of time.
The different types of vasculitis are classified according to the size and location of the blood vessels that are affected.
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Behcet’s commonly involves small to medium sized blood vessels. Males and females are affected equally but males tend to have more severe disease. Behcet’s is rare in the United States. In the US, it is estimated that around 3-5/100,000 people have Behcet’s. In Turkey, where it is very common, as many as 1 in 250 people have it. It is a leading cause of blindness in Israel and Japan.
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Buerger’s or thromboangiitis obliterans is a non-atherosclerotic, segmental, inflammatory and clotting disease that mostly affects the small to medium-sized arteries and veins of the extremities, and leads to decrease or loss of blood supply (ischemia) distally, and in severe cases may cause ulcers and gangrene.
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Central Nervous System
Central nervous system vasculitis (CNSV) is inflammation of blood vessel walls in the brain or spine.
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Cryogloblinemia is a vasculitis of small blood vessels that is caused by deposition of immune complexes: large aggregates of antibodies and the other proteins they are bound to. Some other types of vasculitis are also caused by immune complexes, but cryoglobulinemia is defined by a laboratory test that identifies immune complexes that fall out of solution in the cold. The severity of the disease is highly variable. Skin, joints, and nerves are commonly affected. Kidney disease is somewhat less common and with a wide range of severity. The heart, brain, or gastrointestinal tract each are affected in fewer than 10% of cases.
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Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss Syndrome)
Churg-Strauss Syndrome (CSS), now also referred to by its medically more accurate term eosinophilic granulomatosis with polyangiitis (EGPA), is a rare systemic vasculitis (inflammation in the wall of blood vessels of the body), predominantly affecting small-sized vessels. Because antineutrophil cytoplasmic auto-antibodies (ANCA) can be detected in the serum (liquid and a cellular part of the blood) in up to 40% of the patients, it is also considered one of the three ANCA-associated vasculitides, along with granulomatosis with polyangiitis (Wegener’s) and microscopic polyangiitis. However, the clinical characteristics and outcomes of these three vasculitides differ noticeably.
Learn more about Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome).
Giant Cell Arteritis (Temporal Arteritis)
Giant cell arteritis (GCA) also known as temporal arteritis is an autoimmune disease. Autoimmune diseases are conditions in which immune cells, that normally help fight infections, are misdirected to attack your own tissues. In the case of GCA, these immune cells are involved in an inflammatory reaction in large arteries of the body, mainly branches of the aorta and often, the aorta itself. The inflammation damages the affected blood vessels. It has been estimated that 228,000 Americans have GCA. At present the exact cause of this condition is unknown.
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Granulomatosis with Polyangiitis (Wegener’s)
Granulomatosis with polyangiitis (GPA) (formerly known as Wegener’s granulomatosis) is an uncommon disease affecting the sinuses, nose and lungs and frequently involves the kidneys, lungs, eyes, ears, throat, skin and other body organs.
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Henoch-Schönlein Purpura (HSP) is the most common vasculitis in children and is usually a benign disease that completely resolves in most patients without the need for aggressive treatment. It rarely occurs in adults. It typically presents with skin rash, pain in the abdomen and arthritis.
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Hypersensitivity Vasculitis (HSV) 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.
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Aortitis is a general term which means inflammation of the aorta, the large blood vessel which transports blood from the heart to the body. The aortic root is the part of the aorta which is directly connected to the left ventricle (chamber) of the heart. The blood flows from the aorta through its major branches like the carotid arteries to the head, the subclavian arteries to the arms, and iliac arteries to the legs, as well as other branches to organs like the kidneys, spleen and bowel.
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Kawasaki is a rare vasculitis, which strikes children. In the U.S. over 4,200 children are diagnosed with it each year. 80% of patients are under the age of five. Patients usually begin with a fever that lasts at least five days, despite fever medications. 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.
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Microscopic Polyangiitis (MPA) is a small vessel vasculitis which primarily affects the arterioles (small arteries), capillaries and venules (small veins). Since patients with this condition often have a positive blood test known as an antineutrophil cytoplasmic antibody (ANCA), it is also a form of “ANCA-associated vasculitis”. Granulomatosis with polyangiitis (GPA/Wegener’s) is a related condition that shares features with MPA including the presence of ANCA.
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Polyarteritis Nodosa (PAN) is a form of vasculitis (inflammation in the wall of blood vessels). PAN involves medium-sized and small arteries in the body. It is an autoimmune disease, meaning that the immune system (which normally fights infection) behaves abnormally and causes damage to your own blood vessels.
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Polymyalgia Rheumatica (PMR) is an inflammatory condition which primarily affects large joints and other joint structures surrounding joints such as the bursa. It causes severe stiffness and pain in the shoulders, hips and neck, which is worse in the morning and improves as the day goes on. In this respect, it is related to other forms of inflammatory arthritis, such as rheumatoid arthritis. However, unlike rheumatoid arthritis which can affect virtually every joint in the body outside of the spine, PMR typically does not affect joints other than the shoulders and hips, and PMR can also affect the vertebra structures of the neck. In some cases, patients with PMR may develop inflammation of large blood vessels of the body.
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Rheumatoid Vasculitis (RV) is a serious complication of long standing rheumatoid arthritis (RA) in which inflammation spreads to involve small to medium sized and rarely, large blood vessels in the body. When inflamed, blood vessel walls become thickened and their lumen narrows down, often to the point of complete blockage. This compromises blood supply to the affected organ. Multiple organs may be affected including the skin, nerves, eye, heart, lung, brain, gastrointestinal tract or kidney. It can be life threatening in some circumstances where appropriate treatment is not initiated promptly. Research shows that since the advent of effective drug therapy for RA, fewer people are developing rheumatoid vasculitis in recent years.
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Takayasu arteritis is a rare, chronic inflammation of the large blood vessels, usually the aorta, and its branches. This means the major arteries going to your brain; limbs; heart; lung and kidneys are most likely to be affected. The inflammation can cause narrowing of the vessels (stenosis) or weakening of the vessel wall leading to dilatation and formation of aneurysms (balloon – like enlargements of the vessel wall).
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Urticarial Vasculitis (UV) is a form of vasculitis that affects the skin, causing wheals or hives and/or red patches due to swelling of the small blood vessels. It has two forms: One with normal levels of proteins called complements; the other with low levels of complements; it’s called hypocomplementemic vasculitis.
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