Abstract

Primary biliary cholangitis (PBC) is an autoimmune liver disease characterised by a chronic and destructive lymphocytic cholangitis of the small bile duct, with variable risk of progression.1 Ursodeoxycholic acid (UDCA) is the first-line licensed disease-modifying treatment. Add-on therapy with obeticholic acid (OCA) is recommended for patients who do not respond to UDCA. Moreover, off-label therapy with the pan-PPAR agonist bezafibrate is recognised as an alternative and triple combination therapy -including UDCA, OCA, and fibrates- can normalise biochemical liver tests in difficult-to-treat PBC.

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