Primary sclerosing cholangitis in beta-thalassemia with suspected pigment stone: a diagnostic confusion

Primary sclerosing cholangitis is a chronic inflammatory disease resulting in progressive fibrosis of the biliary tract. The diagnosis is made by demonstrating a beaded appearance in the cholangiography while excluding the secondary causes of sclerosing cholangitis. We report a 27-year-old Sri Lankan female who presented with cholestatic jaundice, whose beta-thalassemia carrier state and pigment stones led to a misdiagnosis as common bile duct obstruction. The magnetic resonance cholangiopancreatography confirmed the diagnosis.