This article was originally published on HCP Live.
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A new systematic review and meta-analysis supports the use of immunosuppressive therapy to achieve native liver survival in children with autoimmune hepatitis in cases of acute liver failure.
New data presented this week at the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) 2023 Annual Meeting in San Diego show that combined treatment with corticosteroids and immunomodulators is associated with improved native liver survival in pediatric patients rate related. Acute liver failure caused by autoimmune hepatitis. These findings may provide clinicians with clearer guidance on intervention strategies that ultimately avoid liver transplantation in younger patients.
Researchers led by Harry Sutton, MD, of the Division of Gastroenterology, Hepatology and Nutrition at the Hospital for Sick Children in Toronto, sought to improve the description and results of treatment strategies and outcomes for pediatric patients with autoimmune hepatitis who suffer from acute liver failure. There is currently a lack of large cohort analysis data for liver studies.
“Autoimmune hepatitis in children has multiple manifestations, but when acute liver failure occurs, it can be fatal and often requires liver transplantation,” the researchers wrote. “Liver transplantation can be avoided with immunosuppressive therapy.”
Sutton and colleagues conducted a systematic review using clinical trial data published between 2000 and 2020 in PubMed and Embase. Eligible trials for their analysis included pediatric and adolescent patients aged <21 years with a diagnosis of type 1 or type 2 autoimmune hepatitis, as defined by serum immunoglobulin levels, autoantibodies, and liver histopathology.
The research team excluded patients with positive hepatitis A-E serology; evidence of drug-induced liver injury; MDR3 deficiency or Wilson disease; history of corticosteroid or nonsteroidal immunomodulator therapy; or liver transplantation not readily available at the reporting clinic receive treatment.
Researchers seek individual patient-level data to explain clinical and biochemical characteristics, prescribed therapies, and clinical outcomes of disease.
Their final analysis included 325 patients from 61 identified studies, as well as 5 patients from their own institution with relevant clinical criteria. The median patient age was 10 years (range 6 – 14 years), and two-thirds (67%) were female. Patients with type 2 autoimmune hepatitis were significantly younger at presentation than their peers with type 1 disease (6.9 years vs 11.0 years; ask =.044).
Regarding treatment outcomes, three out of five patients (n = 197 (60%)) achieved native liver survival. An additional 35% (n = 116) received liver transplantation; 5% (n = 17) died during the evaluation period. Sutton and colleagues observed that pediatric patients with autoimmune hepatitis-related acute liver failure were 2.5 times more likely to achieve native liver survival (odds ratio (OR)) when treated with corticosteroids plus nonsteroidal immunomodulators , 2.5; 95% CI, 1.3 – 5.1; ask =.008).
The researchers also observed that patients with type 2 autoimmune hepatitis had a 3.3-fold increased risk of liver transplantation or death compared with patients with type 1 hepatitis (95% CI, 1.1 to 10.0; ask =.03).
The team noted that their findings were limited by the retrospective nature of the review and analysis. Nonetheless, they concluded that children who first develop autoimmune hepatitis in acute liver failure have a significantly increased likelihood of native liver survival when treated with a combination of corticosteroids and immunosuppression.
“These data raise hope for native liver survival in autoimmune hepatitis-acute liver failure and suggest the need to identify biomarkers to predict which children will require combination immunosuppressants to avoid liver transplantation,” they wrote.