PRIMARY SCLEROSING CHOLANGITIS - REQUIEM FOR BILIARY DRAINAGE OPERATIONS

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.