Minireviews
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Dec 27, 2018; 10(12): 911-923
Published online Dec 27, 2018. doi: 10.4254/wjh.v10.i12.911
Autoimmune hepatitis: Appraisal of current treatment guidelines
Dhruv Lowe, Savio John
Dhruv Lowe, Savio John, Division of Gastroenterology and Hepatology, Department of Medicine, State University of New York, Upstate Medical University, Syracuse, NY 13202, United States
Author contributions: Lowe D performed the literature review and wrote the manuscript; John S co-authored, edited and reviewed the manuscript.
Conflict-of-interest statement: Lowe D and John S do not have any affiliations with or involvement in any organization or entity with any financial or non-financial interest in the subject matter pertaining to this manuscript.
Open-Access: This 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 Non Commercial (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: Savio John, MD, Associate Professor, Division of Gastroenterology and Hepatology, Department of Medicine, State University of New York, Upstate Medical University, 750 E Adams Street, Syracuse, NY 13210, United States. johns@upstate.edu
Telephone: +1-315-4641600 Fax: +1-315-4641601
Received: August 28, 2018
Peer-review started: August 28, 2018
First decision: October 5, 2018
Revised: October 16, 2018
Accepted: October 23, 2018
Article in press: October 23, 2018
Published online: December 27, 2018
Processing time: 121 Days and 12.8 Hours
Abstract

Autoimmune hepatitis affects patients of all ages and gender, across all geographic regions. Although still rare, its incidence and prevalence are increasing. Genetic predisposition conveyed by human leucocyte antigen is a strong risk factor for the disease and may be responsible in part for the wide variation in presentation in different geographic regions. Our understanding of the underlying pathogenic mechanisms is evolving and may lead to development of more targeted immunotherapies. Diagnosis is based on elevated levels of serum aminotransferases, gamma globulins, autoantibodies and characteristic findings on histology. Exclusion of other causes of chronic hepatitis is important. Although undiagnosed disease is associated with poor outcomes, it is readily treatable with timely immunosuppressive therapy in the majority of patients. International guidelines are available to guide management but there exists a disparity in the standard treatment regimens. This minireview aims to review the available guidelines and summarize the key recommendations involved in management of this complex autoimmune disease.

Keywords: Autoimmune hepatitis; Treatment; Hypergammaglobulinemia; Autoantibodies; Azathioprine

Core tip: Autoimmune hepatitis, is a rare inflammatory condition of the liver that can affect all ages and gender, across all geographic regions. It has a wide variability in clinical presentation and thus, diagnosis can be challenging. While undiagnosed disease leads to significant morbidity and mortality, timely initiation of treatment leads to favorable outcomes in the majority of cases. Guidelines are available by international societies but there exists a disparity in the standard treatment indications and regimens. In this minireview, we summarize key points from the available literature and guidelines, focusing on appropriate indications and different treatment regimens available.