Congenital Immunodeficiency Diseases: Crossroad of Infection, Autoimmunity, and Hyperinflammation. Crossroad of Infection, Autoimmunity, and Hyperinflammation

J. Farmer, K. Csomos, R. Bacchetta, J. E. Walter

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Primary immunodeficiency disorders (PIDs) are genetically predetermined conditions of innate or adaptive immune system dysregulation. Susceptibility toward infection has been a long-standing, often diagnosis-requiring, feature. More recently, autoimmunity as a central pathology in PIDs has been described. Our growing ability to link unique genetic defects with downstream mechanisms that drive hyperinflammation and loss of self-tolerance is extremely powerful. Ultimately, successful management of the PID patient requires not only prevention of infection, but early detection and treatment of autoimmune complications through the use of mechanism-based therapies, which will directly address the immune dysregulation at play.

Original languageEnglish
Title of host publicationTranslational Immunology: Mechanisms and Pharmacologic Approaches
PublisherElsevier Inc.
Pages45-81
Number of pages37
ISBN (Print)9780128015773
DOIs
Publication statusPublished - Dec 19 2015

Keywords

  • Autoimmunity
  • Common variable
  • Congenital
  • Hyperinflammation
  • Immunodeficiency
  • Primary

ASJC Scopus subject areas

  • Medicine(all)
  • Immunology and Microbiology(all)

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