Antiproteinase 3- and antimyeloperoxidase-associated vasculitis

Abstract

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.