Review
Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Apr 14, 2017; 23(14): 2459-2469
Published online Apr 14, 2017. doi: 10.3748/wjg.v23.i14.2459
Pathogenesis and clinical spectrum of primary sclerosing cholangitis
Neelam G Gidwaney, Swati Pawa, Kiron M Das
Neelam G Gidwaney, Swati Pawa, Kiron M Das, Division of Gastroenterology and Hepatology, Rutgers-Robert Wood Johnson Medical School, New Brunswick, NJ 08903, United States
Author contributions: Gidwaney NG, Pawa S and Das KM collaborated equally in performing the literature search and writing the review article.
Conflict-of-interest statement: Authors declare no conflict of interests for this article.
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: Kiron M Das, MD, PhD, FACP, FRCP, AGAF, FACG, Chief, Professor of Medicine, Division of Gastroenterology and Hepatology, Rutgers-Robert Wood Johnson Medical School, One Robert Wood Johnson Place, Medical Education Building 478B, New Brunswick, NJ 08903, United States. daskm@rwjms.rutgers.edu
Telephone: +1-732-2356994 Fax: +1-732-2355537
Received: October 24, 2016
Peer-review started: October 28, 2016
First decision: December 19, 2016
Revised: January 21, 2017
Accepted: March 20, 2017
Article in press: March 20, 2017
Published online: April 14, 2017
Core Tip

Core tip: Primary sclerosing cholangitis (PSC) is a fascinating disease with numerous and overlapping theorized pathogenetic models. An autoimmune etiology is in part due to its association with inflammatory bowel disease and autoimmune hepatitis, and inclusion within the IgG4 spectrum of diseases. Though PSC has been documented in the literature for more than a century, only sparse details exist regarding its true pathogenetics and even less about successful medical therapy. More rigorous research is needed to truly understand and treat this disease entity.