Autoimmune liver diseases: assessment of changes in clinical presentation, diagnostic approaches and treatment in two cohorts ten years apart

Background: Autoimmune Liver Diseases (AILDs)— namely Autoimmune Hepatitis (AIH), Primary Biliary Cholangitis (PBC), and Primary Sclerosing Cholangitis (PSC)—are rare chronic disorders characterized by immune-mediated liver injury. Advances in diagnostics, therapeutics, and international registries such as the ERN RARE-LIVER Registry have reshaped their management. However, few studies have evaluated how clinical presentation and treatment have evolved in real-world practice. Aim: To assess temporal changes in the clinical, diagnostic, and therapeutic profiles of AILDs by comparing two patient cohorts approximately ten years apart. Methods: A single-centre, retrospective and prospective study was conducted at the Autoimmune and Cholestatic Liver Disease Clinic, University Hospital of Padua. Patients diagnosed with AIH, PBC, or PSC between 2015–2017 (cohort 15-17) were compared with those prospectively enrolled in the ERN RARE-LIVER Registry between 2023–2025 (cohort 23-25). Demographic, clinical, and therapeutic data were analysed to identify temporal trends. Results: A total of 29 and 30 patients with AIH, 38 and 28 with PBC, and 17 and 16 with PSC were included in the two cohorts, respectively. In AIH, the cohort 23-25 showed lower fibrosis stages (median 1 vs 3, p<0.001) and higher biochemical remission at 6 and 12 months (65% and 100% vs 53% and 68%), with reduced corticosteroid use (22% vs 61%). In PBC, the use of liver biopsy markedly declined in favour of serological diagnosis. Biochemical response to UDCA remained unchanged, but new disease-specific second-line therapies became available. In PSC, the prevalence of IBD remained stable, while fewer cirrhotic cases, transplant referrals, and cholangitis episodes were observed-likely reflecting the shorter follow-up in the recent cohort. Conclusions: Over the past decade, meaningful changes have occurred in the diagnosis and management of AILDs, driven by improved diagnostic tools, novel therapeutic options, and optimized use of established treatments. Continued prospective data collection and standardized approaches are essential to further improve outcomes for patients with these rare but challenging diseases.