Waitlist survival of patients with primary sclerosing cholangitis in the model for end-stage liver disease era.

The ability of the Model for End-Stage Liver Disease (MELD) score to capture the urgency of transplantation may not be generalizable to patients with primary sclerosing cholangitis (PSC) because these patients face unique risks of death or removal from the liver transplant waitlist due to disease-specific complications (eg, repeated bouts of bacterial cholangitis and cholangiocarcinoma). We constructed Cox regression models to determine whether disease-based differences exist in waitlist mortality before liver transplantation. We compared the times to death or withdrawal from the waitlist due to clinical deterioration among patients with or without PSC in the United States after the implementation of the MELD allocation score. Over an 8-year period, 14,073 non-PSC patients (20.5%) and 432 PSC patients (13.6%) died or were removed (P < 0.0001). The adjusted hazard ratio (HR) for PSC was 0.72 [95% confidence interval (CI) = 0.66-0.79], which indicated that these patients had a lower time-dependent risk of death or removal from the waitlist in comparison with patients without PSC. This difference was explained in part by the groups' different probabilities of portal hypertension complications at listing because adjustments for these intermediate endpoints moved the HR closer to the null (0.84, 95% CI = 0.74-0.97). In comparison with patients with other forms of end-stage liver disease, patients with PSC are less likely to die or be removed from the waitlist because of clinical deterioration; therefore, the prevailing practice in some centers and regions of preemptively referring PSC patients for living donor transplantation or exception points should be reconsidered.