Primary sclerosing cholangitis is a chronic cholestatic disease of intrahepatic and extrahepatic biliary ducts, characterised by chronic periductal inflammation and sclerosis of the ducts, which results in segmental stenoses of bile ducts, cholestasis, fibrosis, and, ultimately, liver cirrhosis. Patients with primary sclerosing cholangitis are at higher risk of cholangiocarcinoma as well as of colonic neoplasia, since primary sclerosing cholangitis is associated with inflammatory bowel disease in more than 80% of patients. Several therapeutic modalities have been proposed for primary sclerosing cholangitis, like ursodeoxycholic acid, glucocorticosteroids and immunomodulatory agents, but none has been successful in reversing the process of the disease. To date, liver transplantation is the only definite therapeutic solution for patients with advanced primary sclerosing cholangitis with liver cirrhosis.
Two trials on glucocorticosteroids for primary sclerosing cholangitis were identified. One trial compared biliary lavage with hydrocortisone versus saline. This trial was stopped due to adverse events. The other trial compared oral administration of budesonide versus prednisone. No statistically significant effects were found on mortality, serum activity of alkaline phosphatases, serum bilirubin, and adverse events for any of the evaluated intervention regimens.