Treatment of Primary Sclerosing Cholangitis Including Transplantation

Primary sclerosing cholangitis is a progressive cholestatic liver disease that causes stricturing of the intra and extrahepatic bile ducts that can lead to cirrhosis and end stage liver disease . Effective medical therapy has been elusive, but a course of ursodeoxycholic acid may be prescribed at doses of 17-23 mg/kg/day for up to a year to determine if a reduction in serum alkaline phosphatase is observed. A number of drugs are under investigation, including FXR agonists with choleretic and antimicrobial properties . Liver transplantation for PSC has one of the highest survival rates, but recurrent PSC is seen in up to 25% of recipients.