Background: Liver transplantation has emerged as the definitive treatment for primary sclerosing cholangitis (PSC). Its relationships to inflammatory bowel disease and cholangiocarcinoma were evaluated in this series.
Methods: Fifty-three liver transplantations were performed in 41 patients with PSC at the University of Wisconsin from 1986 through 1994. Fourteen of the patients underwent colectomies for inflammatory bowel disease, eight before transplantation and six after transplantation. Five patients had cholangiocarcinoma on the hepatectomy specimen, and another two had been diagnosed before transplantation.
Results: Patient survival for PSC without cholangiocarcinoma was 85% and 62% at 2 and 9 years, respectively. No patient with PSC and cholangiocarcinoma has survived 2 years, although two patients were free of disease 11 and 20 months after transplantation. Despite maintenance immunosuppression seven patients with liver transplants had reactivation of inflammatory bowel disease and colon carcinoma developed in three after liver transplantation.
Conclusions: Liver transplantation should be performed early in the course of PSC to avoid the lethal complications of cholangiocarcinoma. Careful colonoscopic follow-up is necessary in patients undergoing transplantation for PSC because immunosuppressive therapy does not necessarily cause inflammatory bowel disease to be quiescent, nor does it reduce the risk of colon carcinoma developing.