ArticleGastro hep advances2026
Increased Liver-Related Morbidity in Autoimmune Hepatitis-Primary Sclerosing Cholangitis Variant Syndrome: A Propensity-Matched Multicenter Cohort Study.
Article in Gastro hep advances, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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9 authors.
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Abstract
Background and Aims: Autoimmune hepatitis-primary sclerosing cholangitis (AIH-PSC) variant syndrome is characterized by concurrent hepatitic and cholestatic liver injury, yet data on long-term outcomes relative to both component diseases remain limited. Methods: Using the TriNetX Research Network, we performed 2 separate 1:1 propensity score-matched (PSM) analyses comparing AIH-PSC to AIH alone and to PSC alone, matching on demographics, comorbidities, and baseline laboratory values. A 180-day landmark was applied. Primary outcomes included cirrhosis, hepatocellular carcinoma, liver transplantation, all-cause hospitalization, and long-term steroid use. Cholangiocarcinoma (CCA) was analyzed separately using multivariable Cox regression. Hazard ratios (HRs) served as the primary effect measure. Results: After matching, 985 patients per cohort were included in the AIH comparison and 961 per cohort in the PSC comparison. Compared with AIH alone, AIH-PSC had significantly higher hazards of cirrhosis (HR: 3.138), hepatocellular carcinoma (HR: 2.360), liver transplantation (HR: 4.351), and long-term steroid use (HR: 1.690; all Conclusion: AIH-PSC variant syndrome confers additive liver-related morbidity exceeding either component disease, with significantly higher risks of cirrhosis, hepatobiliary malignancy, liver transplantation, and steroid dependence. CCA risk is driven by the PSC component. These findings support intensified surveillance and early transplant evaluation in this population.
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