Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Nov 28, 2016; 8(33): 1419-1441
Published online Nov 28, 2016. doi: 10.4254/wjh.v8.i33.1419
Recent advances in the diagnosis and treatment of primary biliary cholangitis
Ying-Qiu Huang
Ying-Qiu Huang, Department of Gastroenterology, General Hospital of Benxi Steel and Iron (Group) Co., LTD, Fifth Clinical College of China Medical University, Benxi 117000, Liaoning Province, China
Author contributions: Huang YQ independently wrote the manuscript.
Conflict-of-interest statement: The author declares no 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:
Correspondence to: Ying-Qiu Huang, Professor of Medicine, Chief Physician, Department of Gastroenterology, General Hospital of Benxi Steel and Iron (Group) Co., LTD, Fifth Clinical College of China Medical University, 29 Renmin Road, Pingshan District, Benxi 117000, Liaoning Province, China.
Telephone: +86-24-42215137 Fax: +86-24-42215087
Received: March 31, 2016
Peer-review started: April 5, 2016
First decision: June 12, 2016
Revised: July 26, 2016
Accepted: August 27, 2016
Article in press: August 29, 2016
Published online: November 28, 2016

Primary biliary cholangitis (PBC), formerly referred to as primary biliary cirrhosis, is an infrequent progressive intrahepatic cholestatic autoimmune illness that can evolve into hepatic fibrosis, hepatic cirrhosis, hepatic failure, and, in some cases, hepatocellular carcinoma. The disease itself is characterized by T-lymphocyte-mediated chronic non-suppurative destructive cholangitis and elevated serum levels of extremely specific anti-mitochondrial autoantibodies (AMAs). In this article, we will not only review epidemiology, risk factors, natural history, predictive scores, radiologic approaches (e.g., acoustic radiation force impulse imaging, vibration controlled transient elastography, and magnetic resonance elastography), clinical features, serological characteristics covering biochemical markers, immunoglobulins, infections markers, biomarkers, predictive fibrosis marker, specific antibodies (including AMAs such as AMA-M2), anti-nuclear autoantibodies [such as anti-multiple nuclear dot autoantibodies (anti-sp100, PML, NDP52, anti-sp140), anti-rim-like/membranous anti-nuclear autoantibodies (anti-gp210, anti-p62), anti-centromere autoantibodies, and some of the novel autoantibodies], histopathological characteristics of PBC, diagnostic advances, and anti-diastole of PBC. Furthermore, this review emphasizes the recent advances in research of PBC in terms of therapies, including ursodeoxycholic acid, budesonide, methotrexate, obeticholic acid, cyclosporine A, fibrates such as bezafibrate and fenofibrate, rituximab, mesenchymal stem cells transplant, and hepatic transplant. Currently, hepatic transplant remains the only optimal choice with acknowledged treatment efficiency for end-stage PBC patients.

Keywords: Autoimmune liver diseases, Primary biliary cholangitis, Primary biliary cirrhosis, Diagnosis, Therapy

Core tip: Primary biliary cholangitis (PBC), previously called primary biliary cirrhosis, is an autoimmune non-suppurative inflammatory disease of the bile duct that is usually complicated by intrahepatic cholestasis and intrahepatic bile ductule damage, and eventually leads to liver fibrosis and cirrhosis. This review will focus on the clinical, serological and histopathological characteristics of PBC, as well as the advances in the diagnosis and treatment of the disease.