Journal of Clinical Immunology

, Volume 32, Issue 6, pp 1148–1152 | Cite as

Autoimmunity: Homeostasis of Innate Immunity Gone Awry

  • Hyon Ju Park
  • John P. Atkinson
Key Review Article


Autoimmunity is generally considered to represent an attack on self-tissues mediated by autoantibodies or self-reactive T cells. However, the recent identification of genetic variants in inflammatory diseases provides unequivocal examples of defects in innate immunity leading to autoimmunity. The complement system and DNases will be used as telling examples of how genetic variants in innate immune pathways produce injury directly or by triggering the adaptive immune system. The principles illustrated are likely representative of how other innate immune pathways mediate damage to self.

Direct Tissue Damage by Dysregulated Innate Immunity

Atypical hemolytic uremic syndrome (aHUS): Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) characterized by a triad of hemolytic anemia, thrombocytopenia and acute renal failure and is classified as enteropathic (diarrhea-associated) or non-enteropathic (atypical). Rare heterozygous mutations in a negative regulator...


Autoimmunity innate immune system DNase 



Atypical hemolytic uremic syndrome


Thrombotic microangiopathy


Alternative pathway


Hemolytic uremic syndrome


Hemolysis elevated liver enzymes low platelets


Age-related macular degeneration


Retinal pigment epithelium


Single nucleotide polymorphism


Group A streptococcus


Systemic lupus erythematosus




Three prime repair exonuclease 1


Aicardi-Goutières syndrome


Interferon stimulatory DNA


Toll-like receptor


Genome wide association study


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Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  1. 1.Division of RheumatologyWashington University School of MedicineSt. LouisUSA
  2. 2.Department of MedicineWashington University School of MedicineSt. LouisUSA

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