Liver transplantation is the only effective therapeutic option for patients with end-stage liver disease due to primary sclerosing cholangitis (PSC). In this study, we analyzed a single center's experience with 150 consecutive PSC patients who received 174 liver allografts. Mean follow-up was 55 months. Actuarial patient survival at 1, 2, 5, and 10 years was 93.7%, 92.2%, 86.4%, and 69.8%, respectively, whereas graft survival was 83.4%, 83.4%, 79.0%, and 60.5%, respectively. The main indication for retransplantation was hepatic artery thrombosis, and the major cause of death was severe infection. Patients with PSC had a higher incidence of acute cellular and chronic ductopenic rejection compared to a non-PSC control group. Chronic ductopenic rejection adversely affected patient and graft survival. Biliary strictures, both anastomotic and nonanastomotic, were frequent and occurred in 16.2% and 27.2% of patients, respectively. The incidence of recurrent PSC was 20%. A negative impact on patient survival was not seen in patients with either postoperative biliary strictures or recurrence of PSC. Six patients (4%) had cholangiocarcinoma and 1 patient died related to recurrence of malignant disease. Seventy-eight percent of PSC patients had associated inflammatory bowel disease, most commonly chronic ulcerative colitis, which did not adversely impact patient outcome posttransplantation. Nine patients required proctocolectomy after liver transplantation; 5 because of intractable symptoms related to inflammatory bowel disease and 4 due to the development of colorectal carcinoma/highgrade dysplasia. Our data show that liver transplantation provides excellent long-term patient and graft survival for patients with end-stage PSC. (HEPATOLOGY 1999;30:1121-1127.)
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown cause. 1,2 PSC usually has an insidious onset with patients frequently presenting with persistent elevated levels of serum alkaline phosphatase. The diagnosis is made by cholangiography, which shows stricturing, beading, and irregularities of the intrahepatic and/or extrahepatic biliary system in the absence of other identifi-Abbreviation: PSC, primary sclerosing cholangitis. From the Liver Transplant Unit, Mayo Clinic, Rochester, MN.