Review
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Feb 18, 2016; 8(5): 265-272
Published online Feb 18, 2016. doi: 10.4254/wjh.v8.i5.265
Controversies in the management of primary sclerosing cholangitis
Jeremy S Nayagam, Stephen P Pereira, John Devlin, Phillip M Harrison, Deepak Joshi
Jeremy S Nayagam, John Devlin, Phillip M Harrison, Deepak Joshi, Institute of Liver Studies, King’s College Hospital, London SE5 9RS, United Kingdom
Stephen P Pereira, Institute of Liver and Digestive Health, University College London, London NW3 2PF, United Kingdom
Author contributions: All authors contributed equally to the paper with concept and design of the review, literature review, drafting, critical revision and editing, and approval of the final version.
Conflict-of-interest statement: No potential conflict of 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: Dr. Deepak Joshi, Institute of Liver Studies, King’s College Hospital, Denmark Hill, London SE5 9RS, United Kingdom. d.joshi@nhs.net
Telephone: +44-203-2999000
Received: July 29, 2015
Peer-review started: August 6, 2015
First decision: September 16, 2015
Revised: January 13, 2016
Accepted: January 21, 2016
Article in press: January 22, 2016
Published online: February 18, 2016
Processing time: 198 Days and 6.2 Hours
Abstract

Primary sclerosing cholangitis (PSC) remains a rare but significant disease, which affects mainly young males in association with inflammatory bowel disease. There have been few advances in the understanding of the pathogenesis of the condition and no therapeutics with proven mortality benefit aside from liver transplantation. There remain areas of controversy in the management of PSC which include the differentiation from other cholangiopathies, in particular immunoglobulin G4 related sclerosing cholangitis, the management of dominant biliary strictures, and the role of ursodeoxycholic acid. In addition, the timing of liver transplantation in PSC remains difficult to predict with standard liver severity scores. In this review, we address these controversies and highlight the latest evidence base in the management of PSC.

Keywords: Immunoglobulin G4 related sclerosing cholangitis; Cholangiocarcinoma; Primary sclerosing cholangitis; Liver transplantation; Dominant strictures

Core tip: There have been few advances in therapeutics for primary sclerosing cholangitis (PSC) and there remain areas of controversy in the management of PSC. In this review, we address these controversies, which include the differentiation of PSC from other cholangiopathies, in particular immunoglobulin G4 related sclerosing cholangitis, the management of dominant biliary strictures, the role of ursodeoxycholic acid, and the timing of liver transplantation.