Background: Little information is available on the indications for, and the efficacy and timing of, liver transplantation in patients with primary sclerosing cholangitis (PSC). This issue is particularly relevant because prolonged survival has been reported in patients who do not undergo transplantation.
Methods: Long-term results of therapeutic interventions including liver transplantation was assessed in a representative series of 51 patients. Patient survival was compared with that expected from prognostic models.
Results: Actuarial symptom-free survival rate in patients treated by nontransplantation biliary surgery (n = 23) was 35% at 10 years. Actuarial survival rate from onset of PSC (56% at 10 years) was identical to that expected from the prognostic model. Actuarial patient (n = 28) survival rate 5 years after transplantation was greater than that expected from prognostic models (89% versus 31%; p < 0.001). Previous abdominal surgery was associated with an increased in-hospital mortality rate (p < 0.05). Cumulative actuarial incidence of cancer 5 and 10 years after the onset of PSC was 13% and 31%, respectively.
Conclusions: Liver transplantation improves the prognosis of patients with PSC. Failure to identify patients who will benefit from nontransplantation therapeutic interventions or in whom a cancer will develop, and the risk associated with previous abdominal surgery, suggest that liver transplantation should be indicated early after onset of symptoms.