Review
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. May 21, 2016; 22(19): 4651-4661
Published online May 21, 2016. doi: 10.3748/wjg.v22.i19.4651
Auto immune hepatitis
Nicole MF van Gerven, Ynto S de Boer, Chris JJ Mulder, Carin MJ van Nieuwkerk, Gerd Bouma
Nicole MF van Gerven, Ynto S de Boer, Chris JJ Mulder, Carin MJ van Nieuwkerk, Gerd Bouma, Department of Gastroenterology and Hepatology, Vu University Medical Centre, 1081 HV, Amsterdam, The Netherlands
Author contributions: van Gerven NMF wrote the paper; de Boer YS designed the paper; Mulder CJJ supervised the paper; van Nieuwkerk CMJ supervised the paper; Bouma G supervised the paper; all authors critically read and approved the final manuscript.
Conflict-of-interest statement: All authors declare that they have no competing interest.
Open-Access: This article is an open-access article which 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/
Correspondence to: Chris JJ Mulder, Professor, Department of Gastroenterology and Hepatology, Vu University Medical Centre, De Boelelaan 1118, 1081 HV Amsterdam, The Netherlands. cjmulder@vumc.nl
Telephone: +31-20-4440613 Fax: +31-20-4449406
Received: February 27, 2016
Peer-review started: February 28, 2016
First decision: March 21, 2016
Revised: March 29, 2016
Accepted: April 20, 2016
Article in press: April 20, 2016
Published online: May 21, 2016
Processing time: 80 Days and 10.7 Hours
Abstract

To provide an update of the latest trends in epidemiology, clinical course, diagnostics, complications and treatment of auto immune hepatitis (AIH). A search of the MEDLINE database was performed using the search terms: “auto immune hepatitis”, “clinical presentation”, “symptoms”, “signs”, “diagnosis”, “auto antibodies”, “laboratory values”, “serology”, “histopathology”, “histology”, “genetics”, “HLA genes”, “non-HLA genes”, “environment”, “epidemiology”, “prevalence”, “incidence”, “demographics”, “complications”, “HCC”, “PBC”, “PSC”, “corticosteroid”, “therapy”, “treatment”, “alternative treatment”. English-language full-text articles and abstracts were considered. Articles included reviews, meta-analysis, prospective retrospective studies. No publication date restrictions were applied. AIH is an immune meditated progressive inflammatory liver disease that predominantly affects middle-aged females but may affect people of all ages. The clinical spectrum of AIH is wide, ranging from absent or mild symptoms to fulminant hepatic failure. The aetiology of AIH is still unknown, but is believed to occur as the consequence of an aberrant immune response towards an un-known trigger in a genetically susceptible host. In the absence of a gold standard, diagnosis is based on the combination of clinical, biochemical and histopathological criteria. Immunosuppressive treatment has been the cornerstone of treatment since the earliest description of the disease in 1950 by Waldenström. Such treatment is often successful at inducing remission and generally leads to normal life expectancy. Nevertheless, there remain significant areas of unmet aetiological a clinical needs including fundamental insight in disease pathogenesis, optimal therapy, duration of treatment and treatment alternatives in those patients unresponsive to standard treatment regimens.

Keywords: Auto immune hepatitis; Diagnosis; Liver; Epidemiology; Treatment

Core tip: Autoimmune hepatitis (AIH) is a chronic inflammatory liver disorder of unknown aetiology, which can lead to hepatic failure and premature death when untreated. In AIH there is no existence of a pathognomonic feature and therefore the diagnosis rests on a combination of immunological, biochemical, and histological features together with exclusion of other liver diseases. Due to large heterogeneity of the disease, AIH might be unrecognised. Immunosuppressive treatment has been the cornerstone of treatment. Such treatment is often successful at inducing remission. For most patients life long treatment is indicated. In patients in whom all treatments fail, liver transplantation remains a final option.