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Antiproteinase 3- and antimyeloperoxidase-associated vasculitis Franssen CF; Stegeman CA; Kallenberg CG; Gans RO; De Jong PE; Hoorntje SJ; Tervaert JWKidney Int 2000[Jun]; 57 (6): 2195-206Antiproteinase 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.|Antibodies, Antineutrophil Cytoplasmic/analysis/*immunology[MESH]|Humans[MESH]|Kidney Diseases/immunology[MESH]|Myeloblastin[MESH]|Peroxidase/*immunology[MESH]|Respiratory Tract Diseases/immunology[MESH]|Serine Endopeptidases/*immunology[MESH]|Vasculitis/*immunology/physiopathology[MESH] |
