Lessons from a patient with an unusual hepatic overlap syndrome

This Case Study describes a 24-year-old white male with Crohn's disease that was in remission who presented with cholestasis and hyperbilirubinemia. The patient had normal alanine aminotrasferase and γ-globulin levels, and the results of serological tests for an infectious cause of hepatopathia were negative. The patient was diagnosed with autoimmune hepatitis–primary sclerosing cholangitis overlap syndrome on the background of primary sclerosing cholangitis. Background A 24-year-old white male was diagnosed as having Crohn's disease by clinical, endoscopic and histological assessments, and long-term remission was successfully induced with short-course prednisolone. While the Crohn's disease was still in remission, the patient presented with cholestasis and hyperbilirubinemia but normal alanine aminotransferase levels, negative results for serological tests for infectious causes of hepatopathy and normal γ-globulin levels. Investigations Repeated laboratory and serological tests, ultrasonography, endoscopic retrograde cholangiopancreatographies, CT scan, magnetic resonance cholangiopancreatography and liver biopsies. Diagnosis Autoimmune hepatitis–primary sclerosing cholangitis (AIH–PSC) overlap syndrome on the background of PSC with a rare course of sequential manifestations of the different disease components. Management Combined immunosuppression with prednisolone and treatment with ursodeoxycholic acid (UDCA), endoscopic treatment of dominant stenoses, bile duct stent implantation, and close follow-up.