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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

Introduction

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...

Keywords

Autoimmunity innate immune system DNase 

Abbreviations

aHUS

Atypical hemolytic uremic syndrome

TMA

Thrombotic microangiopathy

AP

Alternative pathway

HUS

Hemolytic uremic syndrome

HELLP

Hemolysis elevated liver enzymes low platelets

AMD

Age-related macular degeneration

RPE

Retinal pigment epithelium

SNP

Single nucleotide polymorphism

GAS

Group A streptococcus

SLE

Systemic lupus erythematosus

DNase1

Deoxyribonuclease1

TREX1

Three prime repair exonuclease 1

AGS

Aicardi-Goutières syndrome

ISD

Interferon stimulatory DNA

TLR

Toll-like receptor

GWAS

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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