Primary Sclerosing Cholangitis: Treatment
Author: mzPOTTER
None of the currently available treatments for primary sclerosing cholangitis, has demonstrated a proven effect by modifying the natural history of disease.
The most common drug used is ursodeoxycholic acid, based primarily on the benefits of this bile acid in cholestatic diseases such as primary biliary cirrhosis. The use of this drug in doses up to 15 mg/kg has shown benefits biochemical (decrease of alkaline phosphatase [FA] and gamma glutamyl transpeptidase [GGT]), but has not shown clear benefits histological or survival of patients.
Prospective studies are ongoing using higher doses (20 to 30 mg/kg). A new derivative of ursodeoxycholic acid called ursodeoxycholic rules seems promising pre-clinical studies.
Liver transplantation is the treatment of choice in cases where the disease has advanced. The transplant indication criteria are similar to those of other chronic liver disease. An additional indication for liver transplantation is the presence of recurrent bacterial cholangitis. The only presence of jaundice is not considered an indication for transplant. A Mayo Clinic score to predict mortality greater than 80% over the next six months is usually accepted as a criterion for enlistment for transplantation.
Forecast
Primary sclerosing cholangitis is a disease usually progressive. The median survival without liver transplantation is 12 years. Survival can be estimated using the best score at the Mayo Clinic (Mayo Clinic Risk Score), a formula that includes age, history of variceal hemorrhage, albumin, bilirubin and SGOT. Sclerosing cholangitis may recur after liver transplantation in 20% of patients.