Hepatic angiosarcoma in a patient treated with obeticholic acid for primary biliary cholangitis

Primary Biliary Cholangitis (PBC) is a chronic cholestatic liver disease that can lead to cirrhosis and the development of hepatocellular carcinoma. PBC is not known to be associated with hepatic angiosarcoma. Second-line treatments for PBC, including obeticholic acid, have had less than a decade of clinical use. We present a case of a patient with PBC treated with obeticholic acid who subsequently developed hepatic angiosarcoma. The patient is now on active surveillance following resection of the angiosarcoma. The development of this rare and aggressive cancer should prompt closer post-marketing surveillance for obeticholic acid.