What is Vasculitis?

Vasculitis refers to the group of disorders having to do with inflammatory destruction of blood vessels. Examples are Wegener's Granulomatosis, Churg-Strauss Syndrome, Kawasaki Disease, and Buerger's Disease.

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Who gets Wegener's Disease?

Patients are divided equally between men and women. The disease can strike at any age, with the peak of the disease in the 4th or 5th decade of life. The cause is unknown.

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How is Wegener's Granulomatosis diagnosed?

Diagnosis is established by clinical and laboratory testing such as the ANCA test (a blood test). Wegener's Disease can often be confirmed by biopsy. Antineutrophil Cytoplasmic Antibody (ANCA) is manufactured by the bodies immune system. ANCA is found in the majority of patients and its level correlates with the disease activity. Since most people with Wegener's Granulomatosis produce ANCA, they usually have a positive ANCA test. ANCA scores will normally increase as symptoms worsen. The ANCA test is also helpful in monitoring treatment, since the amount of ANCA in the blood tends to fall in some patients as the disease goes into remission.

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What is ANCA?

ANCA stands for Anti-Neutrophil Cytoplasmic Antibody test. This test is highly specific for certain autoimmune diseases affecting blood vessels (Churg-Strauss Syndrome, Microscopic Polyangiitis and Wegener's Granulomatosis). It is done by separating out neutrophils (one type of white blood cell) and staining them with a dye that will fluoresce when exposed to ultra-violet light. The illuminated neutrophils are then examined by microscope to determine the character of the fluorescing granules in the neutrophils. More about the ANCA test can be found here ----> http://www.vasculitisfoundation.org/ANCAtest

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What are the treatments for Wegner's Disease?

Treatments vary and are discussed in length in other sections of this website. Another great place to read about actual treatments being used for real life patients are in the Wegener's forums. Having said that, treatment of Wegener's Granulomatosis usually consists of cytoxic agents (Cytoxan) with or without a glucocorticoid (prednisone). Treatment will vary with increased symptoms and involvement of the disease in each individual. You must maintain a good relationship with your team of doctors, and understand their instructions carefully. It is important to have frequent follow ups, even when in remission and not undergoing therapy.

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Is there a cure for Wegener's Granulomatosis?

Unfortunately, there is no known cure for Wegener's Disease. With early diagnosis and proper treatment the disease can be brought into remission. Long term remission can be induced and maintained with the proper medications and frequent follow-ups. Frequent ANCA tests will monitor the disease while Wegeners Granulomatosis is inactive. Treatment can produce symptom free remission periods of 5 to 20 years or more, but it is important to follow up with ANCA tests to catch a relapse at its earliest and most treatable stage.

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