{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T06:31:34Z","timestamp":1793773894791,"version":"4.6.1"},"reference-count":87,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,11,3]],"date-time":"2025-11-03T00:00:00Z","timestamp":1762128000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Primary biliary cholangitis (PBC) is a chronic, cholestatic disease with a female predominance and a long disease duration. The pathogenesis of PBC is still unclear; however, genetic, epigenetic, and environmental factors, alongside immune dysregulation, seem to lead to a dysfunction of the biliary \u2018bicarbonate umbrella\u2019 and increased biliary epithelial cells apoptosis. Ursodeoxycholic acid (UDCA) has been the treatment of choice for PBC since its approval back in 1994; however, a percentage varying from 15-40% of all patients fail to achieve biochemical response or alkaline phosphatase normalization. Obeticholic acid, though promising at first, failed to show benefit after long-term use and was retracted from the market. Two peroxisome proliferator\u2013activated receptor agonists (PPARs) have recently been approved for use in patients with PBC, showing biochemical response in non-responders and improvement of pruritus. However, a substantial percentage of patients fail to achieve serum alkaline phosphatase and bilirubin normalization; as a result, many drugs with different mechanisms of action are in phase 2 or 3 trials. The aim of this review is to present available data regarding PBC treatment and explain the pathogenetic pathway each one targets.<\/jats:p>","DOI":"10.3389\/fimmu.2025.1698833","type":"journal-article","created":{"date-parts":[[2025,11,3]],"date-time":"2025-11-03T06:24:25Z","timestamp":1762151065000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":7,"title":["Primary biliary cholangitis. Treatment options in 2025. 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