Role of ANCA in the pathogenesis of Wegener's granulomatosis: a short review.

The presence of autoantibodies to neutrophil cytoplasmic proteinase-3, usually reported as c-ANCA, is virtually diagnostic for Wegener's granulomatosis and related conditions. These antibodies are highly associated with active systemic disease, and may be implicated in pathogenesis. In vitro, c-ANCA can induce a respiratory burst and degranulation of cytokine-primed neutrophils, leading to lysis of cultured endothelial cells. This and other putative pathogenic mechanisms are reviewed.