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Outcomes and management in paediatric autoimmune hepatitis presenting as acute liver failure: Individual patient data
Aly Fawzy1, Harry Sutton2, Shannon M Vandriel2
1Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
Insights
Autoimmune hepatitis in children presenting with acute liver failure has better outcomes with combined corticosteroid and non-steroid immunomodulator therapy. Type 1 autoimmune hepatitis independently predicts native liver survival, suggesting tailored treatment approaches are crucial.
Area of Science:
- Pediatric Hepatology
- Autoimmune Diseases
- Gastroenterology
Background:
- Autoimmune hepatitis (AIH) presenting as acute liver failure (ALF) in children is a critical condition often necessitating liver transplantation (LTx).
- Limited large-scale studies exist on pediatric AIH-ALF management and outcomes.
- This study addresses this gap through an individual patient data meta-analysis (IPD).
Purpose of the Study:
- To analyze the management strategies and outcomes of pediatric patients with AIH-ALF.
- To identify factors influencing native liver survival (NLS) and the need for LTx.
- To inform clinical decision-making for this vulnerable patient group.
Main Methods:
- A systematic review and IPD meta-analysis was performed, adhering to PRISMA guidelines.
- Data were sourced from PubMed and Excerpta Medica for studies published between 2000 and 2020.
- Included patients were under 21 years with AIH (Type 1 or 2) presenting as ALF, with extracted clinical, biochemical, intervention, and outcome data.
Main Results:
- The analysis included 335 patients (61 studies + 5 from the institution), predominantly female (66.8%) with a median age of 10.
- Native liver survival (NLS) was achieved in 59.7%, 35% underwent LTx, and 5% died pre-LTx.
- Combination therapy (corticosteroids + non-steroid immunomodulators) increased NLS likelihood 2.5-fold; AIH-1 was independently associated with higher NLS (OR 3.8).
Conclusions:
- Treatment with corticosteroids and non-steroid immunomodulators may improve the probability of NLS in pediatric AIH-ALF.
- Treatment strategies should consider patient-specific factors, particularly the type of AIH.
- Further research is needed to identify biomarkers predicting the need for combination immunosuppression to potentially avoid LTx.
Background And Aims:
Autoimmune hepatitis (AIH) in children presenting in acute liver failure (ALF) can be fatal and often requires liver transplantation (LTx). This individual patient data meta-analysis (IPD) aims to examine management and outcomes of this population, given the lack of large cohort studies on paediatric AIH first presenting as ALF (AIH-ALF).
Methods:
A systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Review and Meta-Analyses of IPD statement using PubMed and Excerpta Medica dataBASE, and included English studies published between 2000 and 2020. The study included patients under 21 years of age, diagnosed with type 1 or 2 AIH and presenting with ALF. Data extracted included clinical and biochemical characteristics, interventions, and outcomes.
Results:
Three hundred and thirty eligible patients from 61 studies were identified, with an additional five patients from our institution. The majority were female (66.8%), with a median age of 10. Overall, 59.7% achieved native liver survival (NLS), 35% underwent LTx, and 5% died before LTx. The use of corticosteroids with non-steroid immunomodulators increased the likelihood of NLS by 2.5-fold compared to corticosteroids alone. AIH-1 was associated with 3.3-fold odds for NLS, compared to AIH-2. However, on multivariate analysis, only AIH-1 was identified as an independent predictor for NLS (OR 3.8 [95% CI 1.03-14.2], p = .04).
Conclusion:
While corticosteroids and non-steroid immunomodulators treatment may offer enhanced probability of achieving NLS, treatment regimens for AIH-ALF may need to consider patient-specific factors, especially AIH type. This highlights the potential for NLS in AIH-ALF and suggest a need to identify biomarkers which predict the need for combination immunosuppression to avoid LTx.
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