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.
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