Complement deficiencies in systemic lupus erythematosus

Curr Allergy Asthma Rep. 2014 Jul;14(7):448. doi: 10.1007/s11882-014-0448-2.


The complement system is a major, multifunctional part of innate immunity and serves as a bridge between the innate and adaptive immune systems. It consists of more than 30 distinct proteins that interact with one another in a specific sequence. There are three pathways of complement activation: the classical, the lectin, and the alternative pathways. The three pathways are initiated by distinct mechanisms, but they all generate the same core set of effector molecules. Inherited complete deficiencies in complement components are generally very rare and predispose to infections and autoimmune disease. One of the better described associations is between deficiencies in early classical pathway components and the development of systemic lupus erythematosus. The goal of this review will be to discuss the associations between and the causal mechanisms of complement deficiencies and systemic lupus erythematosus.

Publication types

  • Review

MeSH terms

  • Autoimmunity
  • Complement Activation / immunology
  • Complement System Proteins / deficiency
  • Complement System Proteins / immunology*
  • Cytokines / immunology
  • Humans
  • Immune Tolerance
  • Lupus Erythematosus, Systemic / diagnosis
  • Lupus Erythematosus, Systemic / immunology*
  • Lupus Erythematosus, Systemic / therapy


  • Cytokines
  • Complement System Proteins