Prevalence and outcomes of acute presentations of autoimmune hepatitis: Systematic Review and Meta-Analysis
In brief
About 4 in 10 autoimmune hepatitis patients present acutely, with outcomes varying sharply
A meta-analysis estimated that 39% of patients with autoimmune hepatitis present acutely, though estimates varied substantially across studies. At 90 days, about 79% of patients with acute severe disease were alive without a transplant, compared with 19% of those with acute liver failure. The sharp difference underscores the importance of distinguishing these presentations, but the evidence remains heterogeneous.
- Journal
- Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association (Q1)
- Published
- 27 October 2026
- Study design
- Systematic review of cohort studies
- Evidence level
- Level 2, Moderate (CEBM 2a)
- Authors
- Sunil Taneja, Nikhil Aravind, Rohit Mehtani, Mahesh K Balasundaram, Kannupriya, Alessio Gerussi, et al.
- PMID
- 42946107
- DOI
- 10.1016/j.cgh.2026.10.009
Why clinicians should know about it
- Picked for Immunology and Allergy (paper of the day, 30 October 2026): Clinical outcomes across acute autoimmune hepatitis presentations
Abstract
BACKGROUND AND AIMS: Acute presentations of autoimmune hepatitis (AIH) ranging from acute hepatitis to acute liver failure (ALF) and acute-on-chronic liver failure (ACLF) are diagnostically challenging and clinically heterogeneous. We performed a systematic review and meta-analysis to determine the prevalence of acute AIH presentations and to evaluate transplant-free survival (TFS) across subtypes. METHODS: We searched PubMed, Embase, Web of Science, Scopus, and the Cochrane Library from inception through July 2025 for observational studies reporting acute AIH presentations. Random-effects models generated pooled prevalence and TFS estimates, with a general/unselected-cohort sensitivity analysis performed where feasible. Study quality was assessed using the JBI checklist. RESULTS: Across 10 studies reporting both a total AIH cohort size and a separately quantified acute presentation, the pooled proportion of AIH patients presenting acutely was 39% (95% CI 27%-54.0%; I2=95%). Thirty-four studies were included to describe different acute phenotypes. The pooled prevalence of acute non-severe AIH (ANS-AIH), acute severe AIH (AS-AIH), AIH-associated ALF, acute/severe exacerbation of chronic AIH, and AIH-associated ACLF was 21% (95% CI, 14%-30%; I2=75.6%), 15.0% (95% CI, 9%-23%; I2=79.4%), 3% (95% CI, 1%-9%; I2=90.1%), 15% (95% CI, 9%-25%; I2=91.8%), and 11% (95% CI, 7%-16%; I2=78.5%), respectively. TFS was standardized to 90-day timepoint where feasible. Pooled 90-day TFS was 79% (95% CI 36-96%; I2=0%) for AS-AIH and 19% (11%-31%; I2=0%) for AIH-ALF. Heterogeneity remained substantial across most phenotypes. CONCLUSIONS: Acute presentations are common in AIH and span phenotypes with profoundly different clinical trajectories. While most patients with ANS-AIH, acute/severe exacerbations and AS-AIH achieve favorable outcomes with timely therapy, progression to ALF carries poor TFS.
Abstract as published, via PubMed.
For healthcare professionals. The summary is generated by AI from the published abstract, and the evidence level is assigned automatically from the study design on the Oxford CEBM hierarchy. Neither is medical advice. Read the full paper before changing practice.