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Clinical spectrum of antineutrophil cytoplasmic antibodies

Ronald J Falk, MD
Section Editors
Richard J Glassock, MD, MACP
Gerald B Appel, MD
Deputy Editor
John P Forman, MD, MSc


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 antibodies (ANCA) had been linked to granulomatosis with polyangiitis (Wegener's), abbreviated as 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 testing currently 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 antibody (ANCA) assays are currently 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 2016. | This topic last updated: Feb 1, 2016.
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