Immunodeficiency and autoimmunity: lessons from systemic lupus erythematosus

Trends Mol Med. 2012 Feb;18(2):101-8. doi: 10.1016/j.molmed.2011.10.005. Epub 2011 Dec 15.


Recent evidence suggests that systemic autoimmunity and immunodeficiency are not separate entities, but rather are interconnected processes. Immunodeficiency results from distinct defects of the immune response and primarily presents as infections but also frequently with autoimmune features. Systemic autoimmunity is the combined effect of multiple genetic variations and infectious and immunoregulatory factors that result in dominant autoimmune manifestations, in addition to frequent and opportunistic infections. The overlap in disease manifestations and symptoms suggests that immunodeficiency should be considered in the presence of autoimmunity, and vice versa. In this review, we present the shared or similar aspects of immunodeficiency and autoimmunity using systemic lupus erythematosus as a paradigm and discuss the implications for clinical care.

Publication types

  • Review

MeSH terms

  • Animals
  • Autoimmunity*
  • Complement System Proteins / genetics
  • Complement System Proteins / immunology
  • Genes, MHC Class I
  • Humans
  • Immunologic Deficiency Syndromes / complications*
  • Immunologic Deficiency Syndromes / immunology
  • Lupus Erythematosus, Systemic / complications*
  • Lupus Erythematosus, Systemic / genetics
  • Lupus Erythematosus, Systemic / immunology*
  • Lupus Erythematosus, Systemic / pathology
  • Lymphocytes / immunology
  • Lymphocytes / pathology


  • Complement System Proteins