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

Abstract

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

Materials and methods: 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).

Results: 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). ).

Conclusions: 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.