{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:59:47Z","timestamp":1787029187049,"version":"build-2736575974"},"reference-count":242,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2023,2,2]],"date-time":"2023-02-02T00:00:00Z","timestamp":1675296000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>Primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are rare immune-related cholangiopathies with still poorly explained pathogenesis. Although triggers of chronic inflammation with subsequent fibrosis that affect cholangiocytes leading to obliteration of bile ducts and conversion to liver cirrhosis are unclear, both disorders are regarded to be multifactorial. Different factors can contribute to the development of hepatocellular injury in the course of progressive cholestasis, including (1) body accumulation of bile acids and their toxicity, (2) decreased food intake and nutrient absorption, (3) gut microbiota transformation, and (4) reorganized host metabolism. Growing evidence suggests that intestinal microbiome composition not only can be altered by liver dysfunction, but in turn, it actively impacts hepatic conditions. In this review, we highlight the role of key factors such as the gut\u2013liver axis, intestinal barrier integrity, bile acid synthesis and circulation, and microbiome composition, which seem to be strongly related to PBC and PSC outcome. Emerging treatments and future therapeutic strategies are also presented.<\/jats:p>","DOI":"10.3390\/nu15030760","type":"journal-article","created":{"date-parts":[[2023,2,2]],"date-time":"2023-02-02T03:47:33Z","timestamp":1675309653000},"page":"760","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Selected Aspects of the Intricate Background of Immune-Related Cholangiopathies\u2014A Critical Overview"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7198-4428","authenticated-orcid":false,"given":"Beata","family":"Kasztelan-Szczerbinska","sequence":"first","affiliation":[{"name":"Department of Gastroenterology with Endoscopy Unit, Medical University of Lublin, 8 Jaczewski Street, 20-954 Lublin, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Rycyk-Bojarzynska","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology with Endoscopy Unit, Medical University of Lublin, 8 Jaczewski Street, 20-954 Lublin, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agnieszka","family":"Szczerbinska","sequence":"additional","affiliation":[{"name":"Medical University of Warsaw, 61 Zwirki i Wigury Street, 02-091 Warsaw, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Halina","family":"Cichoz-Lach","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology with Endoscopy Unit, Medical University of Lublin, 8 Jaczewski Street, 20-954 Lublin, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,2,2]]},"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":"982","DOI":"10.1097\/MEG.0b013e32834aa4bb","article-title":"Protein Energy Malnutrition Predicts Complications in Liver Cirrhosis","volume":"23","author":"Huisman","year":"2011","journal-title":"Eur. 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