Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. May 8, 2015; 7(7): 926-941
Published online May 8, 2015. doi: 10.4254/wjh.v7.i7.926
Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy
Treta Purohit, Mitchell S Cappell
Treta Purohit, Mitchell S Cappell, Division of Gastroenterology and Hepatology, William Beaumont Hospital, Royal Oak, MI 48073, United States
Treta Purohit, Mitchell S Cappell, Oakland University William Beaumont School of Medicine, Royal Oak, MI 48073, United States
Author contributions: Both authors contributed equally to this manuscript.
Conflict-of-interest: None for all authors. This paper does not discuss any confidential pharmaceutical data reviewed by Dr. Cappell as a consultant for the United States Food and Drug Administration (FDA) Advisory Committee on Gastrointestinal Drugs.
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: Mitchell S Cappell, MD, PhD, Division of Gastroenterology and Hepatology, William Beaumont Hospital, MOB 602, 3535 W. Thirteen Mile Road, Royal Oak, MI 48073, United States.
Telephone: +1-248-5511227 Fax: +1-248-5515010
Received: December 8, 2014
Peer-review started: December 11, 2014
First decision: December 26, 2014
Revised: February 9, 2015
Accepted: March 5, 2015
Article in press: March 9, 2015
Published online: May 8, 2015
Core Tip

Core tip: Primary biliary cirrhosis (PBC) is an autoimmune, slowly progressive, cholestatic, liver disease characterized by a triad of chronic cholestasis, circulating anti-mitochondrial antibodies, and characteristic liver biopsy findings of nonsuppurative destructive cholangitis and interlobular bile duct destruction. Prominent clinical features include fatigue, pruritis, jaundice, xanthomas, osteoporosis, and dyslipidemia. Ursodeoxycholic acid is the primary therapy. Obtecholic acid and fibrate are promising new, but incompletely tested, therapies. Liver transplantation is the definitive therapy for advanced disease, with about 70% 10-year survival after transplantation. Complications of cirrhosis from advanced PBC include esophageal varices, ascites, spontaneous bacterial peritonitis, hepatorenal syndrome, and hepatoma formation.