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World J Hepatol. Oct 27, 2021; 13(10): 1328-1340
Published online Oct 27, 2021. doi: 10.4254/wjh.v13.i10.1328
Autoimmune hepatitis in genetic syndromes: A literature review
Anna Paola Capra, Emanuele Chiara, Silvana Briuglia
Anna Paola Capra, Silvana Briuglia, Department of Biomedical, Dental, Morphological and Functional Imaging Sciences, University of Messina, Messina 98100, Italy
Emanuele Chiara, Department of Clinical Pathology, University of Messina, Messina 98100, Italy
Author contributions: Capra AP contributed to literature search, table, final revision of the article; Chiara E contributed to literature search, final revision of the article; Briuglia S contributed to the review idea and design, manuscript drafting and final revision of the article.
Conflict-of-interest statement: The authors have no conflicts or financial support to declare.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Silvana Briuglia, MD, Academic Research, Department of Biomedical, Dental, Morphological and Functional Imaging Sciences, University of Messina, Via Consolare Valeria 1, Messina 98100, Italy. silvana.briuglia@unime.it
Received: February 24, 2021
Peer-review started: February 24, 2021
First decision: June 15, 2021
Revised: June 25, 2021
Accepted: September 6, 2021
Article in press: September 6, 2021
Published online: October 27, 2021
Core Tip

Core Tip: Autoimmunity is a relevant health problem, burdened by delay in diagnosis and difficult therapeutic approach. Genetic syndromes often include autoimmune diseases in their typically complex clinical picture. This review explores the association between genetic syndromes and a specific autoimmune disease, autoimmune hepatitis in order to understand if there are pathogenetic mechanisms based on specific mutations, but also how much autoimmune hepatitis is frequent in genetic syndromes. This systematic approach showed an interesting correlation between these two important groups of diseases.