From First Presentation to Transplantation: Risk Factors for Severe Outcome in Pediatric Autoimmune Hepatitis

Our aim was to identify the potential predictors of liver transplant in patients with autoimmune hepatitis based on data at initial presentation.

We retrospectively evaluated records of children diagnosed with autoimmune hepatitis between 2000 and 2025. We excluded 21 patients with de novo autoimmune hepatitis (n =5) and incomplete data (n = 16). We compared demographic, clinical, biochemical, and histopathological data in 2 study groups: transplanted (n = 9) and not transplanted (n = 81).

Of 90 included patients, 63 (70% ) were female. Median age at diagnosis was 115 months (range, 8-215 months). Among included patients, 70 had type 1 autoimmune hepatitis, 13 had type 2 autoimmune hepatitis, and 7 had seronegative autoimmune hepatitis. Nine patients underwent liver transplant (5 with type 1 autoimmune hepatitis, 4 with type 2 autoimmune hepatitis). Rate of transplant was significantly higher in patients with type 2 autoimmune hepatitis (P = . ⁰³ ). Compared with the nontransplant group, patients in the transplant group were more likely to have type 2 autoimmune hepatitis; higher gamma-glutamyl transferase, bilirubin, international normalized ratio, and immunoglobulin levels; and lower albumin and platelet levels. In addition, this group was more likely to have relapsing disease and moderate-to-severe fibrosis at diagnosis. Multivariate analysis showed that moderate-to-severe fibrosis and total bilirubin level were significant predictors of liver transplant (odds ratio: 21.84, 95%CI, 1.34-355.25; P = . ⁰³⁰ and odds ratio: 1.22, 95% CI, 1.02-1.47; P = . ⁰²⁹, respectively). Autoimmune hepatitis type 2 was not found to be associated with outcome (P = . ⁸³⁹). ).

Systematic risk assessment at diagnosis is important to identify high-risk patients who may benefit from close monitoring, early escalation of therapy, and timely referral for transplant evaluation. Tailoring management strategies based on these predictive factors may improve long-term outcomes in pediatric autoimmune hepatitis.