Liver transplantation for primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown aetiology that is progressive in most symptomatic patients, adv ancing toward cirrhosis and liver failure. Liver transplantation is the onl y therapeutic option for patients with end stage liver disease resulting fr om this disorder. The results of transplantation for PSC are excellent with one-year survival rates of 90-97% and five-year survival rates of 80-85%, but are closely related to pre-transplant Child-Pugh stage. Recurrence of P SC after liver transplantation is common, occurring in up to 20% of patient s, but it appears to have little effect on patient survival, as survival of patients with recurrent PSC is similar to that of those without evidence o f recurrence. Cholangiocarcinoma is a catastrophic complication of PSC and as yet no reliable screening method exists. The results of liver transplant ation for patients with clinically apparent cholangiocarcinoma are extremel y poor, however in patients in whom a microscopic tumour is detected in the explanted liver, survival is similar to those transplanted with PSC withou t cholangiocarcinoma. Activity of inflammatory bowel disease (IBD) appears to be more severe after transplantation, especially in units where steroid immunosuppression is withdrawn early. Colon cancer appears within the first few years after transplantation in approximately 7% of patients with IBD w ho are transplanted for PSC. Annual colonoscopy in this population seems pr udent.