PSC is a sclerosing, inflammatory, and obliterative process involving the biliary tree.
Associations: inflammatory bowel disease (90% of cases of PSC-especially ulcerative colitis), autoimmune pancreatitis, AIDS, multifocal fibrosclerosis syndromes.
Pruritus, RUQ pain, jaundice, fever, weight loss, and malaise. Approximately 44% may be asymptomatic at diagnosis. May progress to cirrhosis with portal hypertension.
Transhepatic or endoscopic cholangiograms reveal stenosis and dilation of the intra- and extrahepatic bile ducts.
Cholangiocarcinoma, Caroli disease (cystic dilation of bile ducts), Fasciola hepatica infection, echinococcosis, and ascariasis. IgG4-associated cholangitis.
| TREATMENT | ||
Primary Sclerosing CholangitisNo satisfactory therapy. Cholangitis should be treated as outlined earlier. 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. |