Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Feb 21, 2015; 21(7): 2236-2241
Published online Feb 21, 2015. doi: 10.3748/wjg.v21.i7.2236
Autoimmune hepatitis-primary biliary cirrhosis concurrent with biliary stricture after liver transplantation
Yong-Zhen Kang, Xiao-Ye Sun, Yi-He Liu, Zhong-Yang Shen
Yong-Zhen Kang, First Central clinic of Tianjin Medical University, Tianjin 300192, China
Xiao-Ye Sun, Yi-He Liu, Zhong-Yang Shen, Department of Organ Transplantation, Tianjin First Center Hospital, Tianjin Key Laboratory of Organ Transplantation, Tianjin 300192, China
Author contributions: Sun XY, Kang YZ and Shen ZY proposed the study; Sun XY, Kang YZ and Liu YH collected and analyzed the clinical data; all authors contributed to the design and interpretation of the study; Sun XY was the guarantor.
Supported by the Key Projects of Tianjin Health Bureau, No. 12KG103; and National High Technology Research and Development Program (863 Program) of China, No. 2012AA021001.
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: Dr. Xiao-Ye Sun, Department of Organ Transplantation, Tianjin First Center Hospital, Tianjin Key Laboratory of Organ Transplantation, 24 Fukang Road, Tianjin 300192, China. sxy_tj@163.com
Telephone: +86-22-23626612 Fax: +86-22-23626612
Received: July 28, 2014
Peer-review started: July 29, 2014
First decision: August 15, 2014
Revised: August 28, 2014
Accepted: October 15, 2014
Article in press: October 15, 2014
Published online: February 21, 2015
Processing time: 198 Days and 2.1 Hours
Abstract

Although the development of de novo autoimmune liver disease after liver transplantation (LT) has been described in both children and adults, autoimmune hepatitis (AIH)-primary biliary cirrhosis (PBC) overlap syndrome has rarely been seen in liver transplant recipients. Here, we report a 50-year-old man who underwent LT for decompensated liver disease secondary to alcoholic steatohepatitis. His liver function tests became markedly abnormal 8 years after LT. Standard autoimmune serological tests were positive for anti-nuclear and anti-mitochondrial antibodies, and a marked biochemical response was observed to a regimen consisting of prednisone and ursodeoxycholic acid added to maintain immunosuppressant tacrolimus. Liver biopsy showed moderate bile duct lesions and periportal lymphocytes infiltrating along with light fibrosis, which confirmed the diagnosis of AIH-PBC overlap syndrome. We believe that this may be a case of post-LT de novo AIH-PBC overlap syndrome; a novel type of autoimmune overlap syndrome.

Keywords: Autoimmune liver disease; Autoimmune hepatitis; Primary biliary cirrhosis; Overlap syndrome; Biliary stricture; Liver transplantation

Core tip: We report a 50-year-old man who was diagnosed with autoimmune hepatitis (AIH)-primary biliary cirrhosis (PBC) overlap syndrome 8 years after liver transplantation. His liver function tests became markedly abnormal. Standard autoimmune serological tests were positive for anti-nuclear and anti-mitochondrial antibodies, and a dramatic biochemical response was observed to a regimen consisting of prednisone and ursodeoxycholic acid added to tacrolimus immunosuppression. Liver transplant biopsy showed moderate bile duct lesions and periportal lymphocytes infiltrating along with light fibrosis, which confirmed a diagnosis of AIH-PBC overlap syndrome.