Tip of the iceberg: A comprehensive review of liver disease in Inborn errors of immunity. [Review]

MedStar author(s):
Citation: Hepatology. 2022 Apr 25PMID: 35466407Institution: MedStar Washington Hospital CenterDepartment: Internal Medicine ResidencyForm of publication: Journal ArticleMedline article type(s): Journal Article | ReviewSubject headings: IN PROCESS -- NOT YET INDEXEDYear: 2022ISSN:
  • 0270-9139
Name of journal: Hepatology (Baltimore, Md.)Abstract: Inborn errors of immunity (IEIs) comprise numerous rare, inherited defects of the immune system that affect about 500,000 people in the United States. As advancements in diagnosis through genetic testing and treatment with targeted immunotherapy and bone marrow transplant emerge, increasing numbers of patients survive into adulthood posing fresh clinical challenges. A large spectrum of hepatobiliary diseases now present in those with immunodeficiency diseases, leading to morbidity and mortality in this population. Awareness of these hepatobiliary diseases has lagged the improved management of the underlying disorders, leading to missed opportunities to improve clinical outcomes. This review article provides a detailed description of specific liver diseases occurring in various inborn errors of immunity. A generalized approach to diagnosis and management of hepatic complications is provided and collaboration with hepatologists, immunologists, and pathologists is emphasized as a requirement for optimizing management and outcomes. Copyright This article is protected by copyright. All rights reserved.All authors: Ben Yakov G, Bergerson JRE, Freeman AF, Gewirtz M, Haddad J, Heller T, Kapuria D, Kleiner DE, Koh C, Sharma D, Viana Rodriguez GFiscal year: FY2022Digital Object Identifier: Date added to catalog: 2022-05-11
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Journal Article MedStar Authors Catalog Article 35466407 Available 35466407

Inborn errors of immunity (IEIs) comprise numerous rare, inherited defects of the immune system that affect about 500,000 people in the United States. As advancements in diagnosis through genetic testing and treatment with targeted immunotherapy and bone marrow transplant emerge, increasing numbers of patients survive into adulthood posing fresh clinical challenges. A large spectrum of hepatobiliary diseases now present in those with immunodeficiency diseases, leading to morbidity and mortality in this population. Awareness of these hepatobiliary diseases has lagged the improved management of the underlying disorders, leading to missed opportunities to improve clinical outcomes. This review article provides a detailed description of specific liver diseases occurring in various inborn errors of immunity. A generalized approach to diagnosis and management of hepatic complications is provided and collaboration with hepatologists, immunologists, and pathologists is emphasized as a requirement for optimizing management and outcomes. Copyright This article is protected by copyright. All rights reserved.

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