Primary biliary cholangitis (PBC) is a chronic progressive cholestatic liver disease that is characterized by the destruction of small intrahepatic bile ducts, portal inflammation, and progressive scarring. The cause of PBC is unknown, but it is probably due to an inherited abnormality of immunoregulation. It constitutes the third most common indication for liver transplantation in adults.

The name of this disease was changed from "primary biliary cirrhosis" to "primary biliary cholangitis" in 2014 ³. Patients present with jaundice and high serum bilirubin. Serum anti-mitochondrial antibody (AMA) tests are highly sensitive and specific for PBC (85-100%). In this case, serum total bilirubin was very high (15.6 mg/dL) and anti-nuclear antibody was positive.