Objective: To review the outcome of a consecutive number of patients w
ith primary sclerosing cholangitis (PSC) treated at one institution to
define prognostic variables and determine the influence of surgery on
outcome. Design: Case series of patients with PSC seen in Cape Town,
South Africa, between 1981 and 1991. Setting: Tertiary referral center
. Patients: Thirty-six patients with PSC were studied. Diagnosis was b
ased on cholangiographic findings of multiple strictures of the bile d
ucts together with compatible clinical and biochemical features. Thirt
y-two patients were followed up prospectively for up to 9 years. Main
Outcome Measures: Patient outcome was defined as good (stable or slowl
y progressive disease) or poor (death or liver transplantation). Resul
ts: During the follow-up period, seven patients with PSC died and two
underwent liver transplantation. Actuarial survival at 5 years was 52%
. An increased serum bilirubin concentration was the only variable at
presentation that independently predicted a poor outcome. Cholangiogra
phy was unhelpful in predicting patient outcome. Six patients who deve
loped obstructive jaundice associated with advanced liver disease unde
rwent biliary drainage operations for surgically correctable stricture
s, but this did not seem to prevent progression of the disease. Two pa
tients who progressed to end-stage liver disease went on to have liver
transplantation and were alive with functioning grafts at 7 and 14 mo
nths, respectively. Conclusions: Symptomatic PSC is a progressive diso
rder with a poor prognosis. Our experience suggests that patients with
advanced liver disease caused by PSC should be considered directly fo
r liver transplantation rather than biliary bypass operations.