Predicting Outcomes in Childhood Autoimmune Hepatitis

G Porta et al. J Pediatr 2021; 229: 95-101. Autoimmune Hepatitis: Predictors of Native Liver Survival in Children and Adolescents

This retrospective study enrolled a total of 819 patients, 89.6% with AIH-1 and 10.4% with AIH-2

Key findings:

  • The overall survival was 93.0%, with a native liver survival (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). 
  • Normal C3 levels was associated with longer NLS ( P = .017). The chance of death or liver transplantation during follow-up was 3.4 times greater in patients with C3 level below normal
  • Death or liver transplantation during follow-up was 2.8 times greater in patients with associated sclerosing cholangitis ( P = .046).

My take: This large cohort from Brazil shows that a significant portion of children with AIH do NOT do well, especially if they have associated sclerosing cholangitis.

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