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Wegener's granulomatosis, microscopic polyangiitis, and renal limited vasculitis are associated with circulating anti-neutrophil cytoplasm antibodies and are an important cause of rapidly progressive glomerulonephritis. This review gives an account of recent advances in the understanding of the pathogenesis underlying these conditions and how these may lead to future treatments. Consideration is given to recent clinical trials in the management of anti-neutrophil cytoplasm antibodies (ANCA)-associated vasculitides.

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Abstract. Anti-neutrophil cytoplasm autoantibodies (ANCA) are implicated in the pathogenesis of systemic vasculitis.