Review
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Apr 27, 2020; 12(4): 116-124
Published online Apr 27, 2020. doi: 10.4254/wjh.v12.i4.116
Geoepidemiologic variation in outcomes of primary sclerosing cholangitis
Tej I Mehta, Simcha Weissman, Brian M Fung, James H Tabibian
Tej I Mehta, Department of Medicine, University of South Dakota Sanford School of Medicine, Sioux Falls, SD 57108, United States
Simcha Weissman, Department of Medicine, Hackensack University-Palisades Medical Center, North Bergen, NJ 07047, United States
Brian M Fung, Department of Medicine, Olive View-UCLA Medical Center, Sylmar, CA 91342, United States
James H Tabibian, Department of Medicine, UCLA-Olive View Medical Center, Sylmar, CA 91342, and Health Sciences Clinical Associate Professor, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, United States
Author contributions: Weissman S and Mehta TI assisted with data acquisition, analyses, and manuscript preparation; Weissman S, Mehta TI and Tabibian JH drafted and critically revised the manuscript; Fung BM critically revised the manuscript and provided input regarding methodology; Tabibian JH provided direct supervision and guidance; Weissman S and Tabibian JH are the article guarantors; all authors agree to the final version of this manuscript.
Conflict-of-interest statement: Authors declare no conflict of interests for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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/
Corresponding author: James H Tabibian, MD, PhD, FACP, Department of Medicine, UCLA-Olive View Medical Center, 14445 Olive View Dr 2B-182, Sylmar, CA 91342, United States. jtabibian@dhs.lacounty.gov
Received: February 19, 2020
Peer-review started: February 19, 2020
First decision: March 15, 2020
Revised: March 15, 2020
Accepted: March 24, 2020
Article in press: March 24, 2020
Published online: April 27, 2020
Processing time: 63 Days and 21.3 Hours
Abstract

Primary sclerosing cholangitis (PSC) is a chronic, progressive, hepatobiliary disease characterized by inflammation and fibrosis of the intra- and extra-hepatic bile ducts. Its natural history is one that generally progresses towards cirrhosis, liver failure, cholangiocarcinoma, and ultimately disease-related death, with a median liver transplantation-free survival time of approximately 15-20 years. However, despite its lethal nature, PSC remains a heterogenous disease with significant variability in outcomes amongst different regions of the world. There are also many regions where the outcomes of PSC have not been studied, limiting the overall understanding of this disease worldwide. In this review, we present the geoepidemiologic variations in outcomes of PSC, with a focus on survival pre- and post-liver transplantation as well as the concurrence of inflammatory bowel disease and hepatobiliary neoplasia.

Keywords: Cholangiocarcinoma; Inflammatory bowel disease; Liver transplantation; Geography; Biliary tract; Autoimmune

Core tip: There appears to be considerable geoepidemiologic variation in the outcomes of primary sclerosing cholangitis (PSC). Median liver transplantation-free survival in adults with PSC ranges from 14 to 21 years, depending on geographic region. Post-liver transplantation survival for PSC in North America and Europe appears to be nearly twice that found in Asia. The overall average risk of cholangiocarcinoma among patients with PSC is approximately 400 times that of the general population, occurring in roughly 7%-9% of all patients with PSC. However, these rates vary from region to region, with East Asia having rates roughly three-times higher compared to other regions. Studies from North America, Europe, and Oceania generally report worse clinical outcomes for patients with PSC-inflammatory bowel disease compared to patients with only PSC or inflammatory bowel disease; however, this association is less prominent in studies from Asia.