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Clinical spectrum of antineutrophil cytoplasmic autoantibodies

Ronald J Falk, MD
Peter A Merkel, MD, MPH
Section Editors
Richard J Glassock, MD, MACP
Gerald B Appel, MD
Deputy Editors
Albert Q Lam, MD
Monica Ramirez Curtis, MD, MPH


In 1982, antibodies directed against neutrophil cytoplasmic antigens were first described in patients with pauci-immune glomerulonephritis [1]. These antibodies were initially believed to be associated with Ross River virus infections. By 1985, however, antineutrophil cytoplasmic autoantibodies (ANCA) had been linked to granulomatosis with polyangiitis (GPA) [2]. Within several more years, a relationship among ANCA, GPA, microscopic polyangiitis (MPA), and "renal-limited" vasculitis (pauci-immune glomerulonephritis without evidence of extrarenal disease) had been established [3,4]. ANCA are also present in a substantial subset (approximately 40 percent) of patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss, abbreviated as EGPA) [5]. ANCA testing plays a critical role in the diagnosis and classification of vasculitides, even as debate about their ultimate importance in the pathogenesis and pathophysiology of these conditions continues.

Following a brief discussion of certain technical issues related to the performance of ANCA assays, a review of the disease associations of ANCA and clinical utility of ANCA testing will be presented here. The role of ANCA in the pathogenesis of GPA and related vasculitides is discussed separately. (See "Pathogenesis of granulomatosis with polyangiitis and related vasculitides".)


Two types of antineutrophil cytoplasmic autoantibody (ANCA) assays are in wide use:

Indirect immunofluorescence assay, using alcohol-fixed buffy coat leukocytes

Enzyme-linked immunosorbent assay (ELISA), using purified specific antigens

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Literature review current through: Sep 2017. | This topic last updated: Dec 12, 2016.
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