Crescentic glomerulonephritis: new aspects of pathogenesis

Semin Nephrol. 2011 Jul;31(4):361-8. doi: 10.1016/j.semnephrol.2011.06.007.


This review provides a summary of recent advances in the understanding of crescentic glomerulonephritis, focusing on antineutrophil cytoplasm antibody (ANCA)-associated vasculitis and anti-glomerular basement membrane (anti-GBM) antibody disease. In ANCA-associated vasculitis (AAV), four main conceptual advances are discussed as follows: (1) evidence for the pathogenicity of ANCA, (2) molecular mimicry and the role of infection in AAV, (3) evidence for aberrant T-cell responses and T-cell regulation in AAV, and (4) advances in understanding of genetic predisposition to AAV. In relation to anti-GBM disease we discuss the following: (1) the nature of the Goodpasture autoantigens, (2) T-cell responses and regulation in anti-GBM disease, and (3) human leukocyte antigen and non-human leukocyte antigen genetic associations.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / etiology
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / genetics
  • Antibodies, Antineutrophil Cytoplasmic / immunology
  • Autoantibodies / immunology
  • Glomerulonephritis / etiology*
  • Glomerulonephritis / immunology
  • Humans
  • Lymphocyte Activation
  • Lysosomal-Associated Membrane Protein 2
  • Lysosome-Associated Membrane Glycoproteins / immunology
  • Peroxidase / immunology
  • T-Lymphocytes / immunology


  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies
  • LAMP2 protein, human
  • Lysosomal-Associated Membrane Protein 2
  • Lysosome-Associated Membrane Glycoproteins
  • antiglomerular basement membrane antibody
  • Peroxidase