Complement in antineutrophil cytoplasmic antibody-associated vasculitis

Clin Exp Nephrol. 2013 Oct;17(5):642-645. doi: 10.1007/s10157-012-0700-9. Epub 2012 Nov 23.


Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a group of autoimmune disorders. It was previously assumed that the complement system is not involved in the development of ANCA-associated vasculitis due to its "pauci-immune" feature in renal histology. However, increasing evidence indicates that activation of the complement system, especially via the alternative complement pathway, plays a crucial role in the pathogenesis of ANCA-associated vasculitis. In this brief review, we discuss the evidence, including in vivo, in vitro, and clinical studies, for complement system activation in ANCA-associated vasculitis.

Publication types

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

MeSH terms

  • Animals
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / blood
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / immunology*
  • Antibodies, Antineutrophil Cytoplasmic / blood*
  • Complement Activation*
  • Complement System Proteins / metabolism*
  • Humans
  • Signal Transduction


  • Antibodies, Antineutrophil Cytoplasmic
  • Complement System Proteins