Cell-derived anaphylatoxins as key mediators of antibody-dependent type II autoimmunity in mice

J Clin Invest. 2006 Feb;116(2):512-20. doi: 10.1172/JCI25536.

Abstract

Complement C5a, a potent anaphylatoxin, is a candidate target molecule for the treatment of inflammatory diseases, such as myocardial ischemia/reperfusion injury, RA, and the antiphospholipid syndrome. In contrast, up until now, no specific contribution of C5a and its receptor, C5aR, was recognized in diseases of antibody-dependent type II autoimmunity. Here we identify C5a as a novel key mediator of autoimmune hemolytic anemia (AIHA) and show that mice lacking C5aR are partially resistant to this IgG autoantibody-induced disease model. Upon administration of anti-erythrocyte antibodies, upregulation of activating Fcgamma receptors (FcgammaRs) on Kupffer cells, as observed in WT mice, was absent in C5aR-deficient mice, and FcgammaR-mediated in vivo erythrophagocytosis was impaired. Surprisingly, in mice deficient in FcgammaRI and FcgammaRIII, anti-erythrocyte antibody-induced C5 and C5a production was abolished, demonstrating the existence of a previously unidentified FcgammaR-mediated C5a-generating pathway. These results show that the development of a full-blown antibody-dependent autoimmune disease requires C5a--produced by and acting on FcgammaR--and may suggest therapeutic benefits of C5 and/or C5a/C5aR blockade in AIHA and other diseases closely related to type II autoimmune injury.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anemia, Hemolytic, Autoimmune / genetics
  • Anemia, Hemolytic, Autoimmune / immunology*
  • Animals
  • Antibodies, Monoclonal / immunology
  • Autoimmunity / physiology*
  • Complement C5a / genetics
  • Complement C5a / immunology*
  • Erythrocytes / metabolism
  • Immunoglobulin G / immunology*
  • Kupffer Cells / cytology
  • Kupffer Cells / metabolism
  • Macrophages, Peritoneal / immunology
  • Mice
  • Mice, Inbred C57BL
  • Mice, Inbred NOD
  • Mice, Knockout
  • Phagocytosis / physiology
  • Receptor, Anaphylatoxin C5a / genetics
  • Receptor, Anaphylatoxin C5a / immunology*
  • Receptors, IgG
  • Survival Rate

Substances

  • Antibodies, Monoclonal
  • Immunoglobulin G
  • Receptor, Anaphylatoxin C5a
  • Receptors, IgG
  • Complement C5a