Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Dec 18, 2015; 7(29): 2896-2905
Published online Dec 18, 2015. doi: 10.4254/wjh.v7.i29.2896
Recurrence of autoimmune liver diseases after liver transplantation
Nabiha Faisal, Eberhard L Renner
Nabiha Faisal, Eberhard L Renner, Liver Transplant Program/Multiorgan Transplant Program, University Health Network/Toronto General Hospital, University of Toronto, Toronto, ON M5G 2N2, Canada
Author contributions: Both authors contributed equally regarding conception and design of this review; Faisal N searched and reviewed the literature, and drafted the manuscript; Renner EL reviewed the literature, and revised the manuscript.
Conflict-of-interest statement: None of the authors has a conflict of interest with regards to this review.
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: Eberhard L Renner, MD, FRCP(C), Professor of Medicine, Director GI Transplantation, Liver Transplant Program/Multiorgan Transplant Program, University Health Network/Toronto General Hospital, University of Toronto, 585 University Avenue, 11 PMB 176, Toronto, ON M5G 2N2, Canada.
Telephone: +1-416-3405221 Fax: +1-416-3403126
Received: July 14, 2015
Peer-review started: July 22, 2015
First decision: August 25, 2015
Revised: November 7, 2015
Accepted: December 1, 2015
Article in press: December 2, 2015
Published online: December 18, 2015

Liver transplantation (LT) is the most effective treatment modality for end stage liver disease caused by many etiologies including autoimmune processes. That said, the need for transplantation for autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC), but not for primary sclerosing cholangitis (PSC), has decreased over the years due to the availability of effective medical treatment. Autoimmune liver diseases have superior transplant outcomes than those of other etiologies. While AIH and PBC can recur after LT, recurrence is of limited clinical significance in most, but not all cases. Recurrent PSC, however, often progresses over years to a stage requiring re-transplantation. The exact incidence and the predisposing factors of disease recurrence remain debated. Better understanding of the pathogenesis and the risk factors of recurrent autoimmune liver diseases is required to develop preventive measures. In this review, we discuss the current knowledge of incidence, diagnosis, risk factors, clinical course, and treatment of recurrent autoimmune liver disease (AIH, PBC, PSC) following LT.

Keywords: Recurrent autoimmune hepatitis, Recurrent primary biliary cirrhosis, Recurrent primary sclerosing cholangitis, Liver transplantation, Immunosuppression, Risk factors, Outcomes, Autoimmune liver diseases

Core tip: There is compelling evidence that autoimmune liver disease recur after liver transplantation, with incidence rates ranging from 10% to 50%. Recurrent autoimmune hepatitis and primary biliary cirrhosis do rarely impair patient and graft survival, but may require changing the immunosuppressive regimen, using corticosteroids or adding ursodeoxycholic acid, respectively. In a proportion of patients, recurrent primary sclerosing cholangitis progresses over years to a stage requiring re-transplantation.