title: Long-term results of patients undergoing liver transplantation for primary sclerosing cholangitis.
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.
authors.affiliation: Liver Transplant Unit, Mayo Clinic, Rochester, MN 55905, USA.
authors.forename: I W
authors.initials: IW
authors.lastname: Graziadei
authors.forename: R H
authors.initials: RH
authors.lastname: Wiesner
authors.forename: P J
authors.initials: PJ
authors.lastname: Marotta
authors.forename: M K
authors.initials: MK
authors.lastname: Porayko
authors.forename: J E
authors.initials: JE
authors.lastname: Hay
authors.forename: M R
authors.initials: MR
authors.lastname: Charlton
authors.forename: J J
authors.initials: JJ
authors.lastname: Poterucha
authors.forename: C B
authors.initials: CB
authors.lastname: Rosen
authors.forename: G J
authors.initials: GJ
authors.lastname: Gores
authors.forename: N F
authors.initials: NF
authors.lastname: LaRusso
authors.forename: R A
authors.initials: RA
authors.lastname: Krom
mesh_terms: D002423:Cause of Death; D015209:Cholangitis, Sclerosing / Q000601:surgery*; D005500:Follow-Up Studies; D006084:Graft Rejection / Q000453:epidemiology; D006085:Graft Survival; D006801:Humans; D015994:Incidence; D016031:Liver Transplantation / Q000401:mortality / Q000502:physiology; D011183:Postoperative Complications / Q000145:classification / Q000453:epidemiology*; D012008:Recurrence; D012189:Retrospective Studies; D015996:Survival Rate; D017741:Survivors; D013997:Time Factors