2023 Volume 65 Issue 5 Pages 478-485
Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease with unknown etiology which is characterized by diffuse and multiple fibrosing strictures in the intra- and extra-hepatic bile duct. Liver transplantation is the only curative treatment, and PSC is a known risk factor for cholangiocarcinoma. The Ministry of Health, Labour and Welfare in Japan considers PSC to be an intractable disease. PSC diagnosis is based on characteristic cholangiographic findings. MRCP and EUS are currently preferred to ERCP, as ERCP holds risks of bacterial cholangitis. However, ERCP still plays an important role in cases with dominant strictures to differentiate cholangiocarcinoma and in cases with minimal changes in the intra-hepatic bile duct during early disease stages. Endoscopic treatment is indicated in cases with dominant strictures, with balloon dilation being the first choice of treatment. Long-term stenting should be performed in selected patients because of the high incidence of bacterial cholangitis due to stent occlusion. Pancreato-biliary endoscopy plays a significant role in the diagnosis and treatment of PSC, though endoscopists should understand the risks and the limitations of the procedure.