This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of diseases (granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis), characterized by destruction and inflammation of small vessels. The clinical signs vary and affect several organs, such as the kidney, stomach, intestine, and lung. Skin lesions, such as purpura and urticaria, result when blood from small vessels leaks under the skin. AAV occurs when neutrophils attack small and medium vessels of the body. The underlying reason for this remains unclear. Treatment includes cyclophosphamide, glucocorticoids and other autoimmune drugs such as rituximab.
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