Immunodeficiency and autoimmunity: companions not opposites

Autoimmunity has long been regarded as the polar opposite of immunodeficiency, but clinical and experimental evidence refute this notion. Indeed, numerous inborn or acquired immunodeficiency syndromes are characterized by the development of autoimmune complications in the setting of deficient immune...

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Main Author: David A. Fox
Format: Article
Language:English
Published: American Society for Clinical Investigation 2022-08-01
Series:The Journal of Clinical Investigation
Online Access:https://doi.org/10.1172/JCI162170
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author David A. Fox
author_facet David A. Fox
author_sort David A. Fox
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description Autoimmunity has long been regarded as the polar opposite of immunodeficiency, but clinical and experimental evidence refute this notion. Indeed, numerous inborn or acquired immunodeficiency syndromes are characterized by the development of autoimmune complications in the setting of deficient immune defenses against microbial pathogens. Appreciation that much of the daily business of a healthy immune system is the avoidance of potentially harmful responses to innocuous environmental antigens or components of the host organism helps provide a context for these observations. In this issue of the JCI, Abt and colleagues report on purine nucleoside phosphorylase (PNP) deficiency, exploring the basis for the autoimmune complications that develop in this particular form of T cell immune deficiency and assigning a key role for overactivation of TLR7.
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spelling doaj.art-0255ea57390542219094ae682488b34a2023-11-07T16:19:14ZengAmerican Society for Clinical InvestigationThe Journal of Clinical Investigation1558-82382022-08-0113216Immunodeficiency and autoimmunity: companions not oppositesDavid A. FoxAutoimmunity has long been regarded as the polar opposite of immunodeficiency, but clinical and experimental evidence refute this notion. Indeed, numerous inborn or acquired immunodeficiency syndromes are characterized by the development of autoimmune complications in the setting of deficient immune defenses against microbial pathogens. Appreciation that much of the daily business of a healthy immune system is the avoidance of potentially harmful responses to innocuous environmental antigens or components of the host organism helps provide a context for these observations. In this issue of the JCI, Abt and colleagues report on purine nucleoside phosphorylase (PNP) deficiency, exploring the basis for the autoimmune complications that develop in this particular form of T cell immune deficiency and assigning a key role for overactivation of TLR7.https://doi.org/10.1172/JCI162170
spellingShingle David A. Fox
Immunodeficiency and autoimmunity: companions not opposites
The Journal of Clinical Investigation
title Immunodeficiency and autoimmunity: companions not opposites
title_full Immunodeficiency and autoimmunity: companions not opposites
title_fullStr Immunodeficiency and autoimmunity: companions not opposites
title_full_unstemmed Immunodeficiency and autoimmunity: companions not opposites
title_short Immunodeficiency and autoimmunity: companions not opposites
title_sort immunodeficiency and autoimmunity companions not opposites
url https://doi.org/10.1172/JCI162170
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