Autoimmune Hepatitis: Predictors of Native Liver Survival in Children and Adolescents

Objective: To determine predictors of native liver survival (NLS) in children and adolescents with autoimmune hepatitis (AIH). Study design: The medical records of children and adolescents with AIH were reviewed. A questionnaire was used to collect data on clinical presentation, biochemical and histologic findings, and treatment. Results: A total of 819 patients were included, 89.6% with AIH-1 and 10.4% with AIH-2. The median age (months) at onset was 108 (min 6; max 210; IQR 59). The female sex was predominant (75.8%). The overall survival was 93.0%, with an NLS of 89.9%; 4.6% underwent liver transplantation. The risk of death or liver transplantation during follow-up was 3.2 times greater in patients with AIH-1 (P =.024). Greater levels of aspartate aminotransferase, alanine aminotransferase, serum albumin, platelet, and normal international normalized ratio at the initial presentation were associated with longer NLS (P =.046, P =.006, P <.001, P =.001, and P =.019, respectively). Normal C3 levels was associated with longer NLS (P =.017), with a chance of death or liver transplantation during follow-up being 3.4 times greater in patients with C3 below normal. Death or liver transplantation during follow-up was 2.8 times greater in patients with associated sclerosing cholangitis (P =.046). Complete remission favored NLS (P <.001), with a risk of death or liver transplantation 11.7 times greater for patients not achieving remission. Conclusions: The best predictors of NLS in children and adolescents with AIH were the AIH-2 subtype, a normal C3 at diagnosis, remission during treatment, and normal a cholangiogram during the disease course.