Primary Biliary Cholangitis: Updates in Management and Goals of Treatment

Primary biliary cholangitis (PBC) is a chronic autoimmune disease of the liver characterized by cholestasis and destruction of bile ducts. Goals of treatment include normalization of liver enzymes and prevention of progression to cirrhosis as well as symptom control. The purpose of this review is to summarize the current state of treatment of primary biliary cholangitis.

In 2021, AASLD released a statement recommending fibrates as an off-label alternative for PBC patients with inadequate response or contraindications to approved therapies. Caution should be used when considering OCA in advanced liver disease. Multiple other therapies for PBC are in phase III trials including PPAR agonists and IBAT inhibitors.

There are two FDA-approved treatments for primary biliary cholangitis, ursodeoxycholic acid (UDCA) and obeticholic acid (OCA). Obeticholic acid is used when there is inadequate response to UDCA, and fibrates can also be considered as off-label alternatives in the setting of inadequate response to first-line therapy. Several other classes of drugs are in development for primary biliary cholangitis including multiple in phase III trials.