Review
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Mar 28, 2023; 29(12): 1795-1810
Published online Mar 28, 2023. doi: 10.3748/wjg.v29.i12.1795
Role of autoantibodies in the clinical management of primary biliary cholangitis
Eirini I Rigopoulou, Dimitrios P Bogdanos
Eirini I Rigopoulou, Department of Medicine and Research Laboratory of Internal Medicine, National Expertise Center of Greece in Autoimmune Liver Diseases, General University Hospital of Larissa, Larissa 41110, Greece
Eirini I Rigopoulou, European Reference Network on Hepatological Diseases (ERN RARE-LIVER), General University Hospital of Larissa, Larissa 41110, Greece
Dimitrios P Bogdanos, Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa 41110, Greece
Author contributions: Rigopoulou EI had the original idea, wrote and revised the manuscript; Bogdanos DP wrote and revised the manuscript.
Conflict-of-interest statement: Eirini I Rigopoulou has nothing to declare; Dimitrios P Bogdanos has received lecture Honoria from Enorasis Hellas, Genesis Pharma, Novartis, Euroimmun, Menarini Hellas, Boehringer Ingelheim, Fresenius Kabi; Dimitrios P Bogdnaos has received grants to support the research and educational activities of his Department from Elpen, Boehringer Ingelheim, Demo and Menarini Hellas; Dimitrios P Bogdanos has received travel bursaries to attend scientific meetings and congresses from Werfen, Hospital Line, Pfizer, Elpen, Enorasis Hellas, Novartis and IFT Hellas.
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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Eirini I Rigopoulou, MD, PhD, Professor, Department of Medicine and Research Laboratory of Internal Medicine, National Expertise Center of Greece in Autoimmune Liver Diseases, General University Hospital of Larissa, Mezourlo, Larissa 41110, Greece. eirigopoulou@med.uth.gr
Received: December 5, 2022
Peer-review started: December 5, 2022
First decision: December 19, 2022
Revised: January 4, 2023
Accepted: March 14, 2023
Article in press: March 14, 2023
Published online: March 28, 2023
Abstract

Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease characterized by immune-driven destruction of small intrahepatic bile ducts leading a proportion of patients to hepatic failure over the years. Diagnosis at early stages in concert with ursodeoxycholic acid treatment has been linked with prevention of disease progression in the majority of cases. Diagnosis of PBC in a patient with cholestasis relies on the detection of disease-specific autoantibodies, including anti-mitochondrial antibodies, and disease-specific anti-nuclear antibodies targeting sp100 and gp210. These autoantibodies assist the diagnosis of the disease, and are amongst few autoantibodies the presence of which is included in the diagnostic criteria of the disease. They have also become important tools evaluating disease prognosis. Herein, we summarize existing data on detection of PBC-related autoantibodies and their clinical significance. Moreover, we provide insight on novel autoantibodies and their possible prognostic role in PBC patients.

Keywords: Primary biliary cholangitis, Anti-mitochondrial antibodies, Primary biliary cholangitis-specific antinuclear antibodies, Anti-sp100, Anti-gp210, Prognosis

Core Tip: The diagnosis of primary biliary cholangitis (PBC) relies on the detection of disease-specific autoantibodies, including anti-mitochondrial antibodies and disease-specific antinuclear antibodies targeting sp100 and gp210. In this review, we summarize existing data on detection of PBC-related autoantibodies and their clinical significance. Moreover, we provide insight on novel autoantibodies and their possible prognostic role in PBC patients.