Am J Gastroenterol 1990 Sep;85(9):1136-41

Primary sclerosing cholangitis: the emerging role for liver transplantation

Langnas AN, Grazi GL, Stratta RJ, Wood RP, Marujo W, Markin RS, Donovan J,
Burnett D, Zetterman R, Sorrell M, et al
Department of Surgery, University of Nebraska Medical Center, Omaha
Abstract

Primary sclerosing cholangitis is a progressive liver disease for which
orthotopic liver transplantation is the only curative procedure. Questions exist
regarding the role of temporizing procedures and the timing of transplantation.
During the past 4 yr, we have performed liver transplants in 177 adult
recipients. Twenty-six patients (14.6%) with primary sclerosing cholangitis
received 30 transplants including 12 men and 14 women. The recipients were
examined for a number of preoperative and postoperative variables. The 4-yr
actuarial survival in patients with primary sclerosing cholangitis after
transplantation was 88%. Patients were segregated according to preoperative risk
variables. Twenty patients were low and medium risk, with one death (95%
survival). Three patients were high risk, with two deaths (33% survival). In
conclusion, orthotopic liver transplantation is safe and effective therapy for
primary sclerosing cholangitis. Early referral for transplantation is
recommended to reduce the mortality associated with this procedure in those with
advanced hepatic failure