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  • Review Article
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ANCA-negative pauci-immune crescentic glomerulonephritis

Nature Reviews Nephrology volume 5, pages 313–318 (2009)Cite this article

Abstract

Crescentic glomerulonephritis is a severe form of glomerular injury that is characterized by disruption of the glomerular basement membrane, cellular proliferation within Bowman space, and (often) fibrinoid necrosis. Pauci-immune crescentic glomerulonephritis, so called because it involves little or no glomerular immunoglobulin deposition, is one of the most common causes of rapidly progressive glomerulonephritis. In the majority of patients, pauci-immune crescentic glomerulonephritis is a manifestation of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. However, some patients with pauci-immune crescentic glomerulonephritis lack ANCAs. This Review compares the prevalence, clinical manifestations, histopathology, and outcomes of ANCA-negative pauci-immune crescentic glomerulonephritis with those of ANCA-positive disease. We also discuss the possible pathogenesis of ANCA-negative pauci-immune crescentic glomerulonephritis, paying particular attention to the mechanisms and role of neutrophil activation.

Key Points

  • Approximately 10–30% of patients with pauci-immune crescentic glomerulonephritis lack antineutrophil cytoplasmic antibodies (ANCAs)

  • ANCA-negative pauci-immune crescentic glomerulonephritis might be a distinct disease entity from ANCA-positive pauci-immune crescentic glomerulonephritis

  • In pauci-immune crescentic glomerulonephritis, ANCA-negative patients have fewer extrarenal symptoms than those who are ANCA-positive

  • The existence of differences in renal histopathology and prognosis between ANCA-negative and ANCA-positive pauci-immune crescentic glomerulonephritis remains controversial

  • Neutrophils are thought to have a major role in the pathogenesis of ANCA-negative pauci-immune crescentic glomerulonephritis

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Figure 1: Renal survival of patients in our study with pauci-immune crescentic glomerulonephritis with and without ANCAs.10

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Acknowledgements

This Review was supported by a grant from the Chinese 985 project (985-2-104-113).

Author information

Authors and Affiliations

  1. Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China

    Min Chen & Ming-Hui Zhao

  2. Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, The Netherlands

    Cees G. M. Kallenberg

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  1. Min Chen
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  3. Ming-Hui Zhao
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Correspondence to Ming-Hui Zhao.

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Chen, M., Kallenberg, C. & Zhao, MH. ANCA-negative pauci-immune crescentic glomerulonephritis. Nat Rev Nephrol 5, 313–318 (2009). https://doi.org/10.1038/nrneph.2009.67

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