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Phenotype and prognosis of primary sclerosing cholangitis-autoimmune hepatitis variant syndrome
Mirjam Kolev1, Rahel Bütikofer2, Yasemin Krucker2
1Assistance Publique-Hôpitaux de Paris (AP-HP). Sorbonne Université, Department of Hepatology, Reference Center for Inflammatory Biliary Diseases and Autoimmune Hepatitis CRMR MIVB-H, European Reference Network on Hepatological Diseases, ERN Rare-Liver, Paris, F-75012, France; Department of Visceral Surgery and Medicine, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland; Graduate School for Health Sciences, University of Bern, Bern, Switzerland.
Background And Aims:
A subset of patients with primary sclerosing cholangitis (PSC) exhibits features of autoimmune hepatitis (AIH), referred to as PSC-AIH variant syndrome (VS). Data on the phenotype and prognosis of VS remain limited. We compared clinical characteristics and outcomes of patients with VS and PSC without VS (non-VS).
Methods:
This retrospective cohort study included patients with PSC followed at a tertiary referral center. VS was defined as moderate-to-severe interface hepatitis on liver histology combined with at least one of the following: alanine aminotransferase ≥5 × upper limit of normal (ULN), IgG ≥1.5 × ULN, or smooth muscle antibody titer ≥1:80.
Results:
Twenty-eight patients with VS and 274 non-VS patients were included. At diagnosis, VS patients were younger (median 20 vs. 30 years, p < 0.001), less frequently had inflammatory bowel disease (46.4% vs. 77.7%, p = 0.001), and more often presented with cirrhosis (25% vs. 8%, p = 0.010). Median follow-up was 9.9 years. Adjusted transplant-free survival was similar between groups (HR 0.33, 95% CI 0.11-1.00; p = 0.051). After adjustment, VS was associated with a lower risk of liver-related events (HR 0.27, 95% CI 0.09-0.80; p = 0.019).
Conclusions:
Despite more frequent cirrhosis at diagnosis, patients with VS had comparable transplant-free survival and lower adjusted hazard of liver-related events.
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