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Chen M et al. – Anti–neutrophil cytoplasmic antibody (ANCA)–associated vasculitides (AAV) are a group of autoimmune disorders including Wegener’s granulomatosis (WG), microscopic polyangiitis (MPA), Churg–Strauss syndrome (CSS) and renal–limited vasculitis (RLV). This paper reviews updated information on the pathogenesis of AAV. Together, clinical, in vitro and in vivo data support a pathogenic role for ANCA in AAV, although this role is more evident for myeloperoxidase–ANCA than for PR3–ANCA. The role of anti– LAMP–2 requires further studies.


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