Autoantibodies Against Complement Components in Systemic Lupus Erythematosus - Role in the Pathogenesis and Clinical Manifestations

Lupus. 2017 Dec;26(14):1550-1555. doi: 10.1177/0961203317709347. Epub 2017 May 18.

Abstract

Many complement structures and a number of additional factors, i.e. autoantibodies, receptors, hormones and cytokines, are implicated in the complex pathogenesis of systemic lupus erythematosus. Genetic defects in the complement as well as functional deficiency due to antibodies against its components lead to different pathological conditions, usually clinically presented. Among them hypocomplementemic urticarial vasculitis, different types of glomerulonephritis as dense deposit disease, IgA nephropathy, atypical haemolytic uremic syndrome and lupus nephritis are very common. These antibodies cause conformational changes leading to pathological activation or inhibition of complement with organ damage and/or limited capacity of the immune system to clear immune complexes and apoptotic debris. Finally, we summarize the role of complement antibodies in the pathogenesis of systemic lupus erythematosus and discuss the mechanism of some related clinical conditions such as infections, thyroiditis, thrombosis, acquired von Willebrand disease, etc.

Keywords: Complement antibodies; clinical manifestation; systemic lupus erythematosus.

MeSH terms

  • Apoptosis / immunology
  • Autoantibodies / immunology*
  • Complement System Proteins / immunology*
  • Humans
  • Immune System / immunology
  • Lupus Erythematosus, Systemic / genetics
  • Lupus Erythematosus, Systemic / immunology*
  • Lupus Erythematosus, Systemic / physiopathology
  • Lupus Nephritis / immunology
  • Lupus Nephritis / physiopathology

Substances

  • Autoantibodies
  • Complement System Proteins