Retrospective Cohort Study
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Apr 7, 2018; 24(13): 1410-1418
Published online Apr 7, 2018. doi: 10.3748/wjg.v24.i13.1410
Potential triggering factors of acute liver failure as a first manifestation of autoimmune hepatitis-a single center experience of 52 adult patients
Matthias Buechter, Paul Manka, Falko Markus Heinemann, Monika Lindemann, Hideo Andreas Baba, Martin Schlattjan, Ali Canbay, Guido Gerken, Alisan Kahraman
Matthias Buechter, Paul Manka, Guido Gerken, Alisan Kahraman, Department of Gastroenterology and Hepatology, University Clinic of Essen, Essen 45147, Germany
Paul Manka, Division of Transplantation Immunology and Mucosal Biology, King’s College, London SE59RJ, United Kingdom
Falko Markus Heinemann, Monika Lindemann, Institute of Transfusion Medicine, University Clinic of Essen, Essen 45147, Germany
Hideo Andreas Baba, Martin Schlattjan, Institute of Pathology, University Clinic of Essen, Essen 45147, Germany
Ali Canbay, Department of Gastroenterology, Hepatology, and Infectious Diseases, Otto-von-Guericke University, Magdeburg 39120, Germany
Author contributions: Buechter M analyzed the data and wrote the manuscript; Manka P analyzed the data and performed the statistics; Heinemann FM and Lindemann M performed the HLA-typing; Baba HA and Schlattjan M evaluated the histological specimens; Canbay A and Gerken G treated the patients; Kahraman A treated the patients and designed the study.
Institutional review board statement: This study was reviewed and approved by the ethics committee of the University Clinic of Essen.
Informed consent statement: All patients gave their written informed consent prior to study inclusion.
Conflict-of-interest statement: All authors have nothing to declare.
Data sharing statement: No additional data are available.
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:
Correspondence to: Kahraman Alisan, MD, Associate Professor, Department of Gastroenterology and Hepatology, University Hospital of Essen, Hufelandstr 55, Essen 45122, Germany.
Telephone: +49-201-72384797 Fax: +49-201-7235655
Received: December 6, 2017
Peer-review started: December 6, 2017
First decision: January 18, 2018
Revised: February 21, 2018
Accepted: March 3, 2018
Article in press: March 3, 2018
Published online: April 7, 2018
Research background

Autoimmune hepatitis (AIH) is generally considered to manifest as a chronic liver disease. So far, only limited data are available investigating patients presenting a fulminant acute liver failure as a first manifestation of this autoimmune disorder. The significance of our study was therefore to investigate the circumstances leading to acute liver failure and onset of autoimmune hepatitis.

Research motivation

In the daily clinical setting, the hepatologist is frequently faced with patients demonstrating only a mild elevation of their liver enzymes. Routine work-up of these cases leads finally to the diagnosis of underlying AIH. However, in few cases, one is challenged with patients without any signs of hepatopathy but rapidly developing a life-threatening acute liver failure (ALF) as their first manifestation of AIH. We here presented potential triggering factors which may activate the “autoimmune machinery” leading to ALF.

Research objectives

The main objective of the present study was to gather more information with focus on potential triggering factors leading to acute presentation of AIH with consecutive liver failure. The clinician would be well-advised to accurately document these underlying conditions.

Research methods

In our retrospective cohort study we investigated patients with histologically-proven AIH and further analyzed the patients who presented acute liver failure. Patients’ demographics, laboratory data, immunosuppressive regime, histology, and outcome were documented and studied.

Research results

We were able to identify potential triggering factors in 26/52 (50.0%) of our previously healthy patients presenting ALF as their first manifestation of AIH. These were drug-induced (e.g., non-steroidal anti-inflammatory drugs and antibiotics) ALF (57.7%), virus-induced (Epstein-Barr, Cytomegalovirus and HEV) ALF (30.8%), and surgery in general anesthesia (11.5%), respectively.

Research conclusions

Approximately 9% of our patients were diagnosed with ALF as their initial presentation of AIH which may be potentially induced by drugs, viral infections, and surgery in general anesthesia. Consequently, the clinician would be well-advised to ask his patients for hepato-toxic drugs and accurately document these underlying conditions. Increases of age, MELD-score, and creatinine levels were associated with lethal outcome or need for urgent liver transplantation.

Research perspectives

With our study and findings we hope to further attract the physician’s attention especially in cases of acute liver failure induced by autoimmune hepatitis. In some cases, these disorders may be triggered by drugs and hepato-tropic viruses. We hope that more studies investigating acute liver failure as a first manifestation of AIH will be available in future.