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Disease Definitions

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Wegener’s Granulomatosis (WG)

Wegener’s Granulomatosis (WG) is a rare form of vasculitis (“vasculitis” means inflammation of the blood vessels) in which the small to medium blood vessels are involved and become inflamed, usually affecting the upper and lower respiratory tracts, ears, eyes, kidneys, skin, or peripheral or central nervous system. Musculoskeletal features are also commonly affected. WG is an auto-immune disease, meaning that it attacks its own body tissues, and there is no known cause for the disease. If diagnosed early, treatment can bring about early remission and prevent organ failure.

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Microscopic Polyangiitis (MPA)

Microscopic Polyangiitis (MPA) is a systemic form of vasculitis (“vasculitis” means inflammation of the blood vessels) affecting small blood vessels, usually capillaries, venules, and arterioles, although it occasionally involves medium-sized arteries. MPA frequently affects the lungs and kidneys and peripheral nervous system, and patients often present with variable combinations of renal manifestations, palpable purpura, abdominal pain, cough, and hemoptysis. Once diagnosed, MPA necessitates aggressive treatment with glucocorticoids and immunosuppressive drugs.

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Churg-Strauss Syndrome (CSS)

Churg Strauss Syndrome (CSS)is a rare form of vasculitis (“vasculitis” means inflammation of the blood vessels) which affects small and medium-sized vessels, with a tendency to involve smaller arteries, arterioles, capillaries and venules. The inflammatory process can cause impaired blood flow to various organ systems and cause temporary or permanent damage. Persons of any age may be affected and there is no difference in prevalence by sex. CSS often occurs in the setting of asthma and/or allergic rhinitis (both usually pre-existing). CSS characteristically affects the upper and lower airways and kidneys. Coronary arteritis, myocarditis, and gut involvement is frequent in CSS. The approach to treatment is similar to that provided for WG.

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Polyarteritis Nodosa (PAN)

Polyarteritis Nodosa (PAN) is a rare form of vasculitis (“vasculitis” means inflammation of the blood vessels ) which affects medium-sized or small arteries and can affect patients of any age. The most frequently involved sites are the kidneys, the gastrointestinal tract, the skin, and the nerves. Affected patients may have fever, musculoskeletal symptoms, skin findings of livedo, nodules, ulcers or gangrene, neurological abnormalities, cardiac disease and gastrointestinal vasculitis. The cause is unknown and glucocorticoids are usually effective initial therapy; some patients require cyclophosphamide.

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Takayasu's Arteritis (TAK)

Takayasu’s Arteritis (TAK) i s a rare form of vasculitis (“vasculitis” means inflammation of the blood vessels ) which affects medium and large sized blood vessels , primarily of the aorta (the main blood vessel that leaves the heart) and its branches. Inflammation of the large blood vessels may cause segments of vessels to weaken and stretch, resulting in an aneurysm (weakening of the vessel wall). Inflammation of the large blood vessels can cause narrowed or even completely blocked segments (called an occlusion). The cause of Takayasu’s arteritis is unknown. Narrowing of blood vessels to the arms or legs may cause fatigue, pain or aching due to reduced blood supply. In some patients, decreased blood flow to the intestines may lead to abdominal pain, especially after meals. Decreased blood flow to the kidneys may cause high blood pressure. Decreased blood flow to the heart can cause heart attacks. The standard treatment of Takayasu’s arteritis is with glucocorticoids, such as prednisone or prednisolone; other drugs that affect the immune system are used as well.

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Giant Cell (Temporal) Arteritis (GCA)

Giant Cell Arteritis (GCA), also known as “Temporal Arteritis”, is a rare form of vasculitis (“vasculitis” means inflammation of the blood vessels ) which affects medium and large sized blood vessels. GCA frequently affects the arteries near the temples, although it can involve arteries in just about any part of the body. The inflammation of the involved artery leads to narrowing and sometimes to complete blockage of the blood vessel. This results in the surrounding tissues being deprived of an adequate blood supply. When GCA involves the arteries that supply blood to the eyes, blindness in one or both eyes may develop suddenly. Some of the more common symptoms in GCA include headaches, pain in the jaw or tongue muscles when eating or talking, and tenderness of the scalp over the temples. Once a convincing diagnosis of GCA is assumed, treatment with glucocorticoids should begin immediately.

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Polymyalgia Rheumatica (PMR)

Polymyalgia rheumatica (PMR) is a form of arthritis that affects older persons. It typically affects the shoulders and hips, as well as the neck and torso, causing aching and morning stiffness. PMR can cause puffiness with swelling of the hands and feet, but is different from rheumatoid arthritis. Rheumatoid arthritis can occur in people of any age, and typically causes swelling of the small joints of the hands and feet. Blood tests for rheumatoid arthritis are typically negative in PMR. PMR is often associated with Giant Cell Arteritis (GCA). About 15% of people who have PMR will develop Giant Cell Arteritis (GCA: put in link); therefore, patients with PMR need to be followed by physicians knowledgeable in both disorders. Patients with PMR also need to immediately report new symptoms to their doctors that might indicate the onset of GCA, especially headaches or visual changes. The links between PMR and GCA are of great interest to researchers studying vasculitis. Some investigators think of the two disorders as part of the same disease spectrum, rather than two distinct diseases.

Treatment with glucocorticoids results in prompt improvement of symptoms. The dose of glucocorticoids is lower than those used for GCA, usually between 15 and 20 mg a day initially, and then slowly reducing the dose. No other drugs have been clearly proven to be of benefit in the treatment of PMR. In most patients, the duration of the disease is about 2 ½ years, but this varies greatly in individuals.

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