Natural History and Predictors of Clinical Outcomes in Autoimmune Liver Diseases: A Multicenter Study.

Abstract

Background and aim: Prognosis in autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) has historically been poor. This multicenter retrospective cohort study investigated the natural history and assessed the predictors of outcomes in patients with AIH, PBC, and PSC.

Methods: AIH, PBC, and PSC patients were identified from the state-wide Hepascore and Clinical Outcome cohort. Overall death or transplant, liver-related mortality (liver-related death or transplant), and liver-related decompensation were determined using a population-based data linkage system. Baseline Liver Outcome Score (LOS), Hepascore, and MELD were examined for predicting outcomes.

Results: Two-hundred thirty-seven AIH patients (24% male, median age 56.6 years [range, 14.3-94.0]), 157 PBC patients (8.3% male, median age 60.5 years [range, 25.6-87.1]), and 167 PSC patients (52.7% male, median age 55.6 years [range, 18.4-88.6]) were enrolled. Five-year transplant-free survival was 88% (95%CI: 81-92%) in AIH, 92% (95%CI: 85-96%) in PBC, and 61% (95%CI: 51-69%) in PSC. PSC had a significantly worse overall death or transplant, liver-related mortality, and liver-related decompensation when compared to AIH and PBC (p < 0.0001). LOS was a significant independent predictor of overall death or transplant, liver-related mortality, and liver-related decompensation among patients with AIH and PBC. LOS was a significant independent predictor of overall death or transplant in patients with PSC, and Hepascore was a significant independent predictor of liver-related mortality and liver-related decompensation.

Conclusions: Outcomes for AIH and PBC are excellent but remain poor in PSC. LOS is a predictor of outcomes in autoimmune liver disease.