An occult primary sclerosing cholangitis unmasked by cholangiography: A case report

Primary sclerosing cholangitis (PSC) is a chronic and cholestatic liver disease. Inflammation, fibrosis and destruction of intra and extra-hepatic ducts are the hallmarks of PSC. Another similar immune-mediated cholangiopathy is primary biliary cholangitis (PBC) in which only intrahepatic ducts are affected and approximately 90–95 % of patients are found to be positive for anti-mitochondrial antibody (AMA). We herein present a rare case of a 50-year-old female presenting with arthralgias who was found to have anti-mitochondrial antibody (AMA)-positive PSC; a marker usually found to be exclusive to PBC. This manuscript adds an interesting layer of complexity to the differential diagnosis whereby a diagnosis of PSC should not be excluded in the setting of a positive AMA.