{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T22:38:48Z","timestamp":1788820728385,"version":"build-2803163510"},"reference-count":154,"publisher":"MDPI AG","issue":"23","license":[{"start":{"date-parts":[[2023,12,3]],"date-time":"2023-12-03T00:00:00Z","timestamp":1701561600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Inborn errors of immunity (IEIs) are a heterogeneous group of diverse clinical and genetic phenotypes that have an estimated combined prevalence as high as 1\/1000. Increased risk of frequent, severe, or opportunistic infections is a common feature of IEIs, but there are also diverse immune-mediated, non-infective complications that are associated with significant morbidity and mortality. As patient survival increases, these are becoming more apparent within the liver. Hepatic involvement of IEIs may not only manifest as infections, but also nodular regenerative hyperplasia, granulomatous disease, autoimmune hepatitis and malignancy. As therapeutic options for patients are expanding, with both pharmaceutical treatments as well as haematopoietic stem cell transplant (HSCT), iatrogenic liver injury is increasingly common and important to identify. This review article summarises the spectrum of hepatic complications seen in IEIs, and highlights the challenges of management within this patient cohort, where immunosuppression is poorly tolerated. Early recognition and prompt diagnosis of potential hepatic complications is therefore crucial in ensuring potentially reversible causes are treated, but significant uncertainty remains regarding best practice for many features of immune dysregulation with limited high-quality evidence.<\/jats:p>","DOI":"10.3390\/jcm12237480","type":"journal-article","created":{"date-parts":[[2023,12,3]],"date-time":"2023-12-03T04:59:16Z","timestamp":1701579556000},"page":"7480","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Differential Diagnosis: Hepatic Complications in Inborn Errors of Immunity"],"prefix":"10.3390","volume":"12","author":[{"given":"Emily","family":"Zinser","sequence":"first","affiliation":[{"name":"Department of Allergy and Clinical Immunology, Frimley Health NHS Foundation Trust, Surrey GU16 7UJ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4716-5692","authenticated-orcid":false,"given":"Ky-Lyn","family":"Tan","sequence":"additional","affiliation":[{"name":"Department of Allergy and Clinical Immunology, Frimley Health NHS Foundation Trust, Surrey GU16 7UJ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Da-In","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Allergy and Clinical Immunology, Royal Surrey NHS Foundation Trust, Surrey GU2 7XX, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachael","family":"O\u2019Brien","sequence":"additional","affiliation":[{"name":"Department of Allergy and Clinical Immunology, Frimley Health NHS Foundation Trust, Surrey GU16 7UJ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alison","family":"Winstanley","sequence":"additional","affiliation":[{"name":"Department of Cellular Pathology, University College London Hospitals NHS Foundation Trust, London NW1 2BU, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1736-2756","authenticated-orcid":false,"given":"Patrick","family":"Yong","sequence":"additional","affiliation":[{"name":"Department of Allergy and Clinical Immunology, Frimley Health NHS Foundation Trust, Surrey GU16 7UJ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,12,3]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1473","DOI":"10.1007\/s10875-022-01289-3","article-title":"Human Inborn Errors of Immunity: 2022 Update on the Classification from the International Union of Immunological Societies Expert Committee","volume":"42","author":"Tangye","year":"2022","journal-title":"J. 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