Lessons from a patient with an unusual hepatic overlap syndrome.

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 gamma-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.