2016 Volume 30 Issue 5 Pages 911-916
Sclerosing cholangitis (SC) is classified as follows; primary (PSC), IgG4 related (IgG4-SC), and secondary SC (SSC). Liver transplantation is the only therapy of late-stage PSC, but endoscopic therapy is effective for cases with jaundice and/or biliary infection, to retard the progression to liver failure. Ursodeoxycholic acid and bezafibrate are used, although the efficacy is controversial. In contrast, steroid is defined to be effective for treatment of IgG4-SC. Intractable cases are treated with immunosuppressive agents. Prognosis is mostly well, but a long-term assessment is still to be established. For SSC, underlying diseases are to be managed.