Reshetnyak VI, Vinnitskaya EV, Maev IV. Primary biliary cholangitis: A historical perspective from xanthomatous lesions to modern molecular biology. World J Gastrointest Pathophysiol 2025; 16(2): 107347 [DOI: 10.4291/wjgp.v16.i2.107347]
Corresponding Author of This Article
Vasiliy Ivanovich Reshetnyak, MD, PhD, Full Professor, Department of Propaedeutics of Internal Diseases and Gastroenterology, Russian University of Medicine, No. 20 Delegatskaya Street, Moscow 127473, Russia. vasiliy.reshetnyak@yandex.ru
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Review
Open-Access Policy of This Article
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/
World J Gastrointest Pathophysiol. Jun 22, 2025; 16(2): 107347 Published online Jun 22, 2025. doi: 10.4291/wjgp.v16.i2.107347
Primary biliary cholangitis: A historical perspective from xanthomatous lesions to modern molecular biology
Vasiliy Ivanovich Reshetnyak, Elena Vladimirovna Vinnitskaya, Igor Veniaminovich Maev
Vasiliy Ivanovich Reshetnyak, Igor Veniaminovich Maev, Department of Propaedeutics of Internal Diseases and Gastroenterology, Russian University of Medicine, Moscow 127473, Russia
Elena Vladimirovna Vinnitskaya, Department of Hepatology, Center for Diagnostics and Treatment of Liver Diseases, Moscow Clinical Scientific and Practical Center, Moscow 111123, Russia
Author contributions: Reshetnyak VI developed the conceptualization and design of the study, the selection and analysis of scientific literature, and the authorship, critical checking, and editing of the review; Vinnitskaya EV contributed to the selection and analysis of scientific literature and the writing of the review; Maev IV contributed to the conceptualization and design of the study and the drafting, critical review, and editing of the review; All authors provided approval for the final version of the review.
Conflict-of-interest statement: No potential conflicts of interest.
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: Vasiliy Ivanovich Reshetnyak, MD, PhD, Full Professor, Department of Propaedeutics of Internal Diseases and Gastroenterology, Russian University of Medicine, No. 20 Delegatskaya Street, Moscow 127473, Russia. vasiliy.reshetnyak@yandex.ru
Received: March 25, 2025 Revised: April 5, 2025 Accepted: April 29, 2025 Published online: June 22, 2025 Processing time: 87 Days and 0.5 Hours
Abstract
Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease characterized by damage and loss of the epithelial lining of small intrahepatic bile ducts, leading to ductopenia and cholestasis. In advanced stages, this process results in cirrhosis and liver failure. The disease belongs to cholangiopathies. The review addressed historical questions concerning: The history of the first mention of this disease; how its nomenclature was formed; when specific serological tests were discovered and their importance in the diagnosis of PBC; the history of ursodeoxycholic and other bile acids for the treatment of PBC; and the significance of modern data on impaired bicarbonate production by cholangiocytes in the pathogenesis of PBC.
Core Tip: The present review was devoted to an examination of the historical aspects of primary biliary cholangitis. The initial references to the disease are examined, along with the evolution of its nomenclature. The importance of the development and the evolution of clinical, laboratory, and instrumental methods for the discovery and elucidation of the pathophysiological mechanisms of primary biliary cholangitis is shown.
