{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T20:16:18Z","timestamp":1790972178614,"version":"4.1.0"},"reference-count":101,"publisher":"MDPI AG","issue":"16","license":[{"start":{"date-parts":[[2025,8,16]],"date-time":"2025-08-16T00:00:00Z","timestamp":1755302400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Primary biliary cholangitis (PBC) is a chronic, immune-mediated liver disease characterized by progressive destruction of the small intrahepatic bile ducts, leading to cholestasis, inflammation, and ultimately fibrosis and cirrhosis. This review emphasizes the central role of bile acids in PBC pathogenesis, exploring how disruptions in their synthesis, transport, and detoxification contribute to cholangiocyte damage and disease progression. In addition to discussing the autoimmune features of PBC, including the presence of specific autoantibodies and cellular immune responses, we examine how bile acid dysregulation exacerbates cholestasis and promotes lipid metabolic disturbances. Particular attention is given to the \u201cbicarbonate umbrella\u201d hypothesis, which describes a protective mechanism by which cholangiocytes resist bile acid\u2013induced injury\u2014an essential factor disrupted in PBC. The aim of this review is to summarize current knowledge gaps in the pathophysiology of PBC, with a focus on the role of bile acids not only as key drivers of disease mechanisms, but also as potential biomarkers of disease progression and treatment response.<\/jats:p>","DOI":"10.3390\/ijms26167905","type":"journal-article","created":{"date-parts":[[2025,8,18]],"date-time":"2025-08-18T16:22:33Z","timestamp":1755534153000},"page":"7905","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Primary Biliary Cholangitis: Immunopathogenesis and the Role of Bile Acid Metabolism in Disease Progression"],"prefix":"10.3390","volume":"26","author":[{"given":"Mar\u00eda","family":"Del Barrio","sequence":"first","affiliation":[{"name":"Gastroenterology and Hepatology Department, Clinical and Traslational Research in Digestive Diseases, Valdecilla Research Institute (IDIVAL), Marqu\u00e9s de Valdecilla University Hospital, 39008 Santander, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3610-2263","authenticated-orcid":false,"given":"\u00c1lvaro","family":"D\u00edaz-Gonz\u00e1lez","sequence":"additional","affiliation":[{"name":"Gastroenterology and Hepatology Department, Clinical and Traslational Research in Digestive Diseases, Valdecilla Research Institute (IDIVAL), Marqu\u00e9s de Valdecilla University Hospital, 39008 Santander, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0934-2202","authenticated-orcid":false,"given":"Marta","family":"Alonso-Pe\u00f1a","sequence":"additional","affiliation":[{"name":"Gastroenterology and Hepatology Department, Clinical and Traslational Research in Digestive Diseases, Valdecilla Research Institute (IDIVAL), Marqu\u00e9s de Valdecilla University Hospital, 39008 Santander, Spain"},{"name":"Departamento de Anatom\u00eda y Biolog\u00eda Celular, Universidad de Cantabria, 39011 Santander, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,8,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1989","DOI":"10.1136\/gutjnl-2020-322362","article-title":"Recent Advances in Clinical Practice: Epidemiology of Autoimmune Liver Diseases","volume":"70","author":"Trivedi","year":"2021","journal-title":"Gut"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1016\/j.medcli.2017.12.021","article-title":"Colangitis Biliar Primaria","volume":"151","year":"2018","journal-title":"Med. 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