Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Aug 21, 2018; 24(31): 3513-3520
Published online Aug 21, 2018. doi: 10.3748/wjg.v24.i31.3513
Osteoporosis in primary biliary cholangitis
Christopher J Danford, Hirsh D Trivedi, Konstantinos Papamichael, Elliot B Tapper, Alan Bonder
Christopher J Danford, Hirsh D Trivedi, Konstantinos Papamichael, Alan Bonder, Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, Unites States
Elliot B Tapper, Department of Hepatology, University of Michigan, Ann Arbor, MI 48109, Unites States
Author contributions: Danford CJ and Trivedi HD performed the literature search and drafting of the manuscript; all authors participated in the conceptual design of the paper and revision of the manuscript.
Supported by NIH T32 training grant, No. 4T32GM103702-04 to Trivedi HD.
Conflict-of-interest statement: There are no other financial disclosures or conflicts of interest in the production of this manuscript.
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: Alan Bonder, MD, Attending Doctor, Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, 110 Francis St. Suite 8E, Boston, MA 02215, United States.
Telephone: +1-617-6321070 Fax: +1-617-6231065
Received: June 1, 2018
Peer-review started: June 1, 2018
First decision: July 6, 2018
Revised: July 11, 2018
Accepted: July 22, 2018
Article in press: July 22, 2018
Published online: August 21, 2018

Primary biliary cholangitis (PBC) is an autoimmune cholestatic liver disease with multiple debilitating complications. Osteoporosis is a common complication of PBC resulting in frequent fractures and leading to significant morbidity in this population, yet evidence for effective therapy is lacking. We sought to summarize our current understanding of the pathophysiology of osteoporosis in PBC, as well as current and emerging therapies in order to guide future research directions. A complete search with a comprehensive literature review was performed with studies from PubMed, EMBASE, Web of Science, Cochrane database, and the Countway Library. Osteoporosis in PBC is driven primarily by decreased bone formation, which differs from the increased bone resorption seen in postmenopausal osteoporosis. Despite this fundamental difference, current treatment recommendations are based primarily on experience with postmenopausal osteoporosis. Trials specific to PBC-related osteoporosis are small and have not consistently demonstrated a benefit in this population. As it stands, prevention of osteoporosis in PBC relies on the mitigation of risk factors such as smoking and alcohol use, as well as encouraging a healthy diet and weight-bearing exercise. The primary medical intervention for the treatment of osteoporosis in PBC remains bisphosphonates though a benefit in terms of fracture reduction has never been shown. This review outlines what is known regarding the pathogenesis of bone disease in PBC and summarizes current and emerging therapies.

Keywords: Biliary cirrhosis, Cholestatic liver disease, Osteopenia, Hepatic osteodystrophy, Bisphosphonates, Hormone replacement therapy

Core tip: This article reviews the available literature on the pathophysiology and management of osteoporosis in primary biliary cholangitis (PBC). PBC-related osteoporosis is driven mainly by decreased bone formation as opposed to the increased bone resorption seen in postmenopausal osteoporosis. Despite this and a lack of evidence of efficacy, bisphosphonates remain the cornerstone of treatment. Future attention should be given to the use of anabolic bone agents in the treatment of PBC-related osteoporosis.