The Child-Pugh classification as a prognostic indicator for survival in primary sclerosing cholangitis.

Clinical decisions in primary sclerosing cholangitis (PSC) depend upon understanding its variable natural history. Several prognostic models for survival have been developed. We explored the Child-Pugh Classification (CPC) to determine if it predicts survival as well as a disease-specific model (DSM). We identified 208 PSC patients who satisfied established criteria. Prognostic variables were measured from the first visit. Kaplan-Meier survival estimates were obtained for CPC twice, once censoring observations at orthotopic liver transplantation and again considering orthotopic liver transplantation as a failure. A stepwise Cox proportional hazards-model was used to identify the factors associated with mortality. For 208 patients, the mean follow-up was 70 months (SD + 55). Kaplan-Meier 7-year survivals for CPC A, B, and C were 89.8%, 68%, and 24.9%, respectively (P < .001). The Cox model identified CPC and age as the most significant predictors of mortality. Adding the DSM risk score did not significantly improve the fit of the model (P = .75). The following were concluded: 1) CPC is a powerful predictor of survival in PSC; 2) DSM does not enhance the predictive ability of CPC; and 3) CPC may be used as an alternative to the DSM in research studies and clinical decision-making.