Elsevier

Kidney International

Volume 57, Issue 6, June 2000, Pages 2195-2206
Kidney International

Perspectives in Renal Medicine
Antiproteinase 3- and antimyeloperoxidase-associated vasculitis

https://doi.org/10.1046/j.1523-1755.2000.00080.xGet rights and content
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Antiproteinase 3- and antimyeloperoxidase-associated vasculitis. 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.

Keywords

ANCA
necrotizing crescentic glomerulonephritis
proteinase 3
myloperoxidase
systemic vasculitis
Wegener's granulomatosis

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