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Anti-neutrophil cytoplasmic (ANCA) and anti-glomerular basement membrane (GBM) autoantibodies in necrotizing and crescentic glomerulonephritis

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Abstract

Anti-neutrophil cytoplasmic autoantibodies (ANCA) and anti-glomerular basement membrane (GBM) necrotizing and crescentic glomerulonephritis are aggressive and destructive glomerular diseases that are associated with and probably caused by circulating ANCA and anti-GBM antibodies. These necrotizing lesions are manifested by acute nephritis and deteriorating kidney function often accompanied by distinctive clinical features of systemic disease. Prompt diagnosis requires clinical acumen that allows for the prompt institution of therapy aimed at removing circulating autoantibodies and quelling the inflammatory process. Continuing exploration of the etiology and pathogenesis of these aggressive inflammatory diseases have gradually uncovered new paradigms for the cause of and more specific therapy for these particular glomerular disorders and for autoimmune glomerular diseases in general.

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Authors and Affiliations

  1. UNC Kidney Center, 7011-E Burnett Womack, Chapel Hill, NC, 27599-7155, USA

    Sofia Lionaki & Ronald J. Falk

  2. Department of Pathology and Lab Medicine, Chapel Hill, NC, USA

    J. Charles Jennette

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  1. Sofia Lionaki
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  2. J. Charles Jennette
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Correspondence to Sofia Lionaki.

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Lionaki, S., Jennette, J.C. & Falk, R.J. Anti-neutrophil cytoplasmic (ANCA) and anti-glomerular basement membrane (GBM) autoantibodies in necrotizing and crescentic glomerulonephritis. Semin Immunopathol 29, 459–474 (2007). https://doi.org/10.1007/s00281-007-0093-0

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