{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T11:26:54Z","timestamp":1772796414058,"version":"3.50.1"},"reference-count":83,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2024,11,30]],"date-time":"2024-11-30T00:00:00Z","timestamp":1732924800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JPM"],"abstract":"<jats:p>Background\/Objectives: Primary biliary cholangitis (PBC) is an autoimmune chronic cholestatic disease of the liver that symptomatically can present with pruritus and fatigue. Its established first- and second-line therapies are ursodeoxycholic acid (UDCA) and obeticholic acid (OCA) although they provide limited symptom management. Liver transplantation offers a potentially curative therapeutic option in refractory cases progressing to cirrhosis. Novel research published after the current guidelines highlights the importance of providing an up-to-date analysis of treatment options available. Methods: In this study, we conducted a literature search using Pubmed, Ovid Medline, and SCOPUS to provide a narrative review of first-line, second-line, and emerging therapies in PBC. Results: UDCA has been well established as a long-term, safe therapy within the literature although it is possible that treatment dosage can be further optimised in refractory patients. It has a favourable side effect profile. Despite improving biochemical markers, histopathological profile, and overall outcomes, up to 30\u201340% of patients are refractory to it. Age and sex are highlighted as independent indicators of non-responsiveness. This necessitates effective second-line therapies. Future trials could aim to investigate UDCA as a co-first-line therapy. Further supporting results for OCA were found in the interim extension trial of the seminal POISE study. The long-term phase 4 COBOLT trial is still awaiting results to further assess the complications, adherence, and potential adverse effects. It is a viable option in UDCA-refractory patients. The high incidence rate of dose-related pruritis indicates that alternative second-line options are needed. Bezafibrate is an off-label antilipemic agent that shows promise as a prospective second-line therapy option. The landmark BEZURSO trial alleviated some efficacy and safety concerns, but it remains associated with elevated serum creatinine; thus, it should be considered with caution. Other prospective second-line therapies are budesonide, triple therapy, and novel agents such as seladelpar and elafibranor. Conclusions: UDCA should remain the treatment of choice for PBC, though perhaps not as monotherapy. With further investigation, BF shows promise as a new second-line therapy alongside OCA, which it may outperform.<\/jats:p>","DOI":"10.3390\/jpm14121133","type":"journal-article","created":{"date-parts":[[2024,12,2]],"date-time":"2024-12-02T05:43:43Z","timestamp":1733118223000},"page":"1133","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Up-to-Date Snapshot of Current and Emerging Medical Therapies in Primary Biliary Cholangitis"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-7407-941X","authenticated-orcid":false,"given":"Zakary","family":"Warsop","sequence":"first","affiliation":[{"name":"Gastroenterology and Hepatology Department, Imperial College London, London SW7 2AZ, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2670-2145","authenticated-orcid":false,"given":"Nikhil","family":"Anand","sequence":"additional","affiliation":[{"name":"Gastroenterology and Hepatology Department, Imperial College London, London SW7 2AZ, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Husam","family":"Al Maliki","sequence":"additional","affiliation":[{"name":"Gastroenterology and Hepatology Department, Imperial College London, London SW7 2AZ, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1449-3307","authenticated-orcid":false,"given":"Shuell","family":"De Souza","sequence":"additional","affiliation":[{"name":"Gastroenterology and Hepatology Department, Imperial College London, London SW7 2AZ, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7461-4983","authenticated-orcid":false,"given":"Arya","family":"Kamyab","sequence":"additional","affiliation":[{"name":"Gastroenterology and Hepatology Department, Imperial College London, London SW7 2AZ, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amin","family":"Al Hadad","sequence":"additional","affiliation":[{"name":"Healthpoint Hospital, Abu Dhabi 112308, United Arab Emirates"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6340-8244","authenticated-orcid":false,"given":"Laith","family":"Alrubaiy","sequence":"additional","affiliation":[{"name":"Healthpoint Hospital, Abu Dhabi 112308, United Arab Emirates"},{"name":"Department of Medicine Health and Life Sciences, Singleton Bay Campus, Swansea University School of Medicine, Swansea SA2 8PP, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,11,30]]},"reference":[{"key":"ref_1","first-page":"1615","article-title":"Pathogenesis of primary biliary cirrhosis","volume":"56","author":"Jones","year":"2007","journal-title":"Gut"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1055\/s-0034-1383730","article-title":"The new epidemiology of primary biliary cirrhosis","volume":"34","author":"Griffiths","year":"2014","journal-title":"Semin. 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