{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T16:48:38Z","timestamp":1792514918466,"version":"4.5.0"},"reference-count":95,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2026,1,30]],"date-time":"2026-01-30T00:00:00Z","timestamp":1769731200000},"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, cholestatic disease, with a female predominance and a female-to-male ratio of approximately 10:1, that typically follows a slowly progressive, decades-long disease course. The disease is usually asymptomatic at the time of diagnosis and it is not uncommon for a patient to present with cirrhosis. Patients with PBC may also present with extrahepatic manifestations, including pruritus, chronic fatigue, and osteoporosis, while co-existence of other autoimmune diseases, such as autoimmune hepatitis, Hashimoto\u2019s disease, Sjogren\u2019s syndrome, or systemic sclerosis is not uncommon. The exact pathogenesis of PBC remains elusive with a variety of different factors, including genetic, epigenetic, and environmental ones, alongside immune dysregulation leading to a dysfunction of biliary \u201cbicarbonate umbrella\u201d, a protective mechanism by which cholangiocyte-secreted bicarbonate creates an alkaline microenvironment shielding the epithelium from bile acid-induced injury, and increased biliary epithelial cells apoptosis.<\/jats:p>","DOI":"10.3390\/ijms27031388","type":"journal-article","created":{"date-parts":[[2026,1,30]],"date-time":"2026-01-30T11:09:22Z","timestamp":1769771362000},"page":"1388","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Primary Biliary Cholangitis Pathogenesis: A Pathophysiology-Based Narrative Review"],"prefix":"10.3390","volume":"27","author":[{"given":"Klairi","family":"Papachristou","sequence":"first","affiliation":[{"name":"Department of Pathophysiology, Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Angelara","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology, Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9844-2813","authenticated-orcid":false,"given":"Konstantinos","family":"Manganas","sequence":"additional","affiliation":[{"name":"First Department of Propaedeutic and Internal Medicine, \u201cLaiko\u201d Hospital, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2556-6230","authenticated-orcid":false,"given":"Theodoros","family":"Androutsakos","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology, Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,1,30]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1620","DOI":"10.1002\/hep.28140","article-title":"Changing Nomenclature for PBC: From \u2018Cirrhosis\u2019 to \u2018Cholangitis\u2019","volume":"62","author":"Beuers","year":"2015","journal-title":"Hepatology"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1261","DOI":"10.1056\/NEJMra043898","article-title":"Primary Biliary Cirrhosis","volume":"353","author":"Kaplan","year":"2005","journal-title":"N. 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