Vasculitis is defined by inflammation of the blood-vessel walland forms the pathological foundation of a diverse group ofindividual disease entities. Among the primary systemic vasculiticdiseases, Wegener's granulomatosis and microscopic polyangiitisshare several common features, including pulmonary capillaritis,pauci-immune focal crescentic necrotizing glomerulonephritis,and circulating antineutrophil cytoplasmic antibodies (ANCA)that are specific for proteinase 3 or myeloperoxidase. Becauseboth diseases are highly associated with ANCA, they are sometimescollectively referred to as ANCA-associated vasculitis. However,the presence of granulomatous inflammation in Wegener's granulomatosisrepresents a potentially important distinction between thesetwo rare diseases, and it remains unclear whether . . . [Full Text of this Article]
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From the Immunologic Diseases Section, Laboratory of Immunoregulation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md.
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