Hepatology 1999 Nov;30(5):1121-7

Long-term results of patients undergoing liver transplantation for primary sclerosing cholangitis

Graziadei IW, Wiesner RH, Marotta PJ, Porayko MK, Hay JE, Charlton MR, Poterucha
JJ, Rosen CB, Gores GJ, LaRusso NF, Krom RA
Liver Transplant Unit, Mayo Clinic, Rochester, MN 55905, USA.

Abstract
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/high-grade
dysplasia. Our data show that liver transplantation provides excellent long-term
patient and graft survival for patients with end-stage PSC