[Complement deficiencies and human diseases]

Ann Biol Clin (Paris). 2014 May-Jun;72(3):271-80. doi: 10.1684/abc.2014.0961.
[Article in French]

Abstract

The complement system is a complex system involving serum and membrane proteins interacting in a regulated manner. The complement system plays a major role in antibacterial immunity, in inflammation, and in immune complex processing. Therefore, deficiencies in complement proteins are associated with increased susceptibility to bacterial infections and autoimmune diseases. These deficiencies can be inherited or acquired. Most of them can be screened by simple laboratory tests but require a diagnosis in a specialized laboratory. All sequences of complement genes are known, and the discovery of a deficiency must lead to genetic testing. The discovery of a congenital deficiency requires a familial study and a prophylaxis. In this article, we review the complement cascade, the laboratory tests to explore it, and the main diseases associated with complement deficiencies.

Keywords: angioedema; autoimmune diseases; complement; immune deficiencies; renal diseases.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Autoimmune Diseases / immunology
  • Bacterial Infections / immunology
  • Complement Activation / immunology
  • Complement System Proteins / deficiency*
  • Complement System Proteins / immunology
  • Disease Susceptibility / immunology
  • Humans
  • Immunologic Deficiency Syndromes / immunology

Substances

  • Complement System Proteins

Supplementary concepts

  • Immune Deficiency Disease