Treatment: Primary Sclerosing Cholangitis

No satisfactory therapy. Cholangitis should be treated as outlined above. Cholestyramine may control pruritus. Supplemental vitamin D and calcium may retard bone loss. Glucocorticoids, methotrexate, and cyclosporine have not been shown to be effective. Ursodeoxycholic acid improves liver tests but has not been shown to affect survival. Surgical relief of biliary obstruction may be appropriate but has a high complication rate. Liver transplantation should be considered in pts with end-stage cirrhosis. Median survival: 9-12 years after diagnosis, with age, bilirubin level, histologic stage, and splenomegaly being predictors of survival.

For a more detailed discussion, see Greenberger NJ, Paumgartner G: Diseases of the Gallbladder and Bile Ducts, Chap. 369, p. 2075, in HPIM-19.