RESUMO
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by progressive fibrosis and destruction of intra- and extrahepatic bile ducts resulting in hepatic failure and death. Only the liver transplantation is the possible treatment for patients to survive. There has been a few reports that steroid is an effective treatment in autoimmune variant sclerosing cholangitis, which is thought to be a familial diseases with different etiology, and steroid responsive biliary strictures be named as immunoglobulin G4 (IgG4)-associated cholangitis (IAC). There is no reliable data regarding effective steroid treatment in autoimmue variant sclerosing cholangitis in Korea. We report a case of 32-year-old male with sclerosing cholangitis, who was diagnosed by endoscopic retrograde cholangiopancreatography (ERCP) and liver biopsy, showing favorable response to prednisolone therapy.