Journal
KIDNEY INTERNATIONAL
Volume 57, Issue 6, Pages 2195-2206Publisher
NATURE PUBLISHING GROUP
DOI: 10.1046/j.1523-1755.2000.00080.x
Keywords
ANCA; necrotizing crescentic glomerulonephritis; proteinase 3; myloperoxidase; systemic vasculitis; Wegener's granulomatosis
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Wegener's granulomatosis, microscopic polyangiitis, and idiopathic pauci-immune necrotizing crescentic glomerulonephritis (NCGN) are strongly associated with antineutrophil cytoplasmic autoantibodies (ANCAs) directed against either proteinase 3 (anti-PR3) or myeloperoxidase (anti-MPO). This has led some investigators to prefer combining these diseases under the common heading of ANCA-associated vasculitides. However, it is increasingly recognized that there are characteristic differences between patients with anti-PR3 and those with anti-MPO-associated vasculitis. This review focuses on the clinical, histopathologic, and possibly pathophysiologic differences between anti-PR3- and anti-MPO-associated vasculitis. Although there is considerable overlap, the anti-PR3- and anti-MPO-associated vasculitides are each characterized by particular clinical and histopathological findings. Extrarenal organ manifestations and respiratory tract granulomas occur more frequently in patients with anti-PR3 than in those with anti-MPO. Anti-PR3-positive patients with NCGN generally have a more dramatic deterioration of their renal function compared with anti-MPO-positive patients. The term ANCA-associated vasculitis is considered as a useful concept in the presence of systemic vasculitis. Likewise, in the presence of vasculitis, the terms anti-PR3-associated vasculitis and anti-MPO-associated vasculitis are useful concepts.
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