Hepatology 1995 Aug;22(2):451-7

Liver transplantation for sclerosing cholangitis

Narumi S, Roberts JP, Emond JC, Lake J, Ascher NL
Department of Surgery, University of California, San Francisco 94143-0780, USA 

Abstract
The clinical course of 37 patients who underwent 46 liver transplantations for
primary (n = 33) and secondary (n = 4) sclerosing cholangitis was reviewed. The
median follow-up was 37 months. The patient and graft survivals for patients
with primary sclerosing cholangitis at 1, 2, and 5 years were 96.9%, 91.6%,
87.9%, and 83.1%, 74.2%, 65.2%, respectively. In the patients with primary
sclerosing cholangitis (PSC), prior surgery except for simple cholecystectomy
was associated with significantly greater operative time and blood loss. No
cholangiocarcinoma was identified at the time of transplantation. Human
leukocyte antigen typing for PSC patients was heavily weighed toward B8 (58.8%)
compared with control (11.8%). Sixty-two percent of patients with PSC also had
inflammatory bowel disease. Moderate or severe rejection requiring OKT3, "rescue
therapy" with FK506, or retransplantation was relatively higher in patients with
inflammatory bowel disease (70%) versus patients without inflammatory bowel
disease (36.4%) and a matched control group (37.5%). Progressive inflammatory
bowel disease was seen in 6 of 19 patients, with 3 developing cancer and a
dysplasia. Two patients in the entire group died of sepsis and 3 of colon cancer
(2 recurrent and 1 primary). These data demonstrate that excellent survival
results can be achieved in this group of patients. Rejection is frequent and
often severe and steroid refractory. Colon cancer represents the most frequent
cause of death in PSC patients after liver transplantation and demands constant
attention