Primary Biliary Cholangitis: personalizing second-line therapies.

Hepatology

Department of Medicine, Division of Gastroenterology and Hepatology, University of California Davis School of Medicine, Sacramento, California, USA.

Published: November 2024

AI Article Synopsis

  • Primary biliary cholangitis (PBC) is an autoimmune disease primarily affecting middle-aged women, characterized by damage to small bile ducts, leading to liver issues like cholestasis and cirrhosis.
  • The main treatment for PBC has been ursodeoxycholic acid, which helps slow disease progression but doesn't alleviate symptoms; new second-line therapies are being developed for patients who don't respond well.
  • Recent medications like obeticholic acid, elafibranor, and seladelpar show promise in improving liver function and reducing cholestasis, while considerations for personalized treatment based on patient features are essential.

Article Abstract

Primary biliary cholangitis (PBC) is an enigmatic, autoimmune disease targeting the small intralobular bile ducts resulting in cholestasis and potentially progression to biliary cirrhosis. Primarily affecting middle-aged women, the diagnosis of PBC is typically straightforward, with most patients presenting with cholestatic liver tests and the highly specific antimitochondrial antibody. For decades, the foundational treatment of PBC has been ursodeoxycholic acid, which delays disease progression in most patients but has no impact on PBC symptoms. Large cohort studies of patients with PBC have established the benefit of maximizing the reduction in serum alkaline phosphatase levels with ursodeoxycholic acid and the need to add second-line agents in patients who do not achieve an adequate response. Advances in the understanding of bile acid physiology have led to the development of new agents that improve cholestasis in patients with PBC and are predicted to reduce the risk of disease progression. Obeticholic acid, the first second-line therapy to be approved for PBC, significantly improves liver biochemistries and has been associated with improved long-term clinical outcomes but is limited by its propensity to induce pruritus. Elafibranor and seladelpar are peroxisome proliferator-activated receptor agonists recently approved for use in patients with PBC, whereas bezafibrate and fenofibrate are available as off-label therapies. They also have shown biochemical improvements among patients with an inadequate response to ursodeoxycholic acid but may improve symptoms of pruritus. Herein, we review the patient features to consider when deciding whether a second-line agent is indicated and which agent to consider for a truly personalized approach to PBC patient care.

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http://dx.doi.org/10.1097/HEP.0000000000001166DOI Listing

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