https://pubmed.ncbi.nlm.nih.gov/36708813/ Autoimmune hepatitis

J Hepatol. 2023 Jan 25;S0168-8278(23)00025-9.
doi: 10.1016/j.jhep.2023.01.013. Online ahead of print.

Long-term outcomes of patients with type 1 or 2 autoimmune hepatitis presenting in childhood

Giuseppe Maggiore 1, Olivier Bernard 2, Antonella Mosca 3, Eric Ballot 4, Catherine Johanet 4, Emmanuel Jacquemin 5

Abstract
Background & aims: In children with autoimmune hepatitis, issues include the particularities of type 2 hepatitis regarding outcome, the possibility of and criteria for attempting withdrawal of treatment and the long-term outcome. We report our experience on these issues.

Methods: From 1973 to 2002, 117 children with type 1 (n=65) or type 2 (n=52) hepatitis, excluding fulminant hepatitis, were treated, primarily with prednisone and azathioprine. Median follow-up was 20 years in survivors.

Results: Normalization of transaminase and prothrombin ratio were observed in 93% and 84% of children, respectively; sustained remission after treatment withdrawal was recorded in 24% of the entire population with a median follow-up of 7 years. Based on lasting normal aminotransferase only and without histology, sustained treatment-free remission was obtained in 11 of 24 children with follow-ups of 4-22 years. Gastrointestinal bleeding from varices and the emergence of extrahepatic autoimmune disorders occurred in 10 and 22 patients, respectively. Liver transplantation was performed in 23 patients at a median age of 21 years. The 30-year probabilities of overall survival and native liver survival were 81% and 61%. No differences were observed between types 1 and 2 hepatitis for any of the component parts of outcome. In the multivariate analysis, a persistent abnormal prothrombin ratio was combined with worse probabilities of overall and native liver survivals.

Conclusions: In terms of liver outcome, type 2 hepatitis is not different from type 1. Withdrawal of treatment is possible without prior liver histology. A persistent abnormal prothrombin ratio identifies patients who will require liver transplantation in adolescence or early adulthood.

Impact and implications: In children with autoimmune hepatitis, there are conflicting reports on the differences between type 1 and type 2 hepatitis regarding outcome, and on the possibility of treatment withdrawal, before which liver histology is required; data concerning >10-year overall and native liver survivals are limited. In this study we found no differences between types 1 and 2 hepatitis; a durable treatment-free state was achieved in 19% of all patients throughout childhood and early adulthood, and in 45% of children for whom treatment withdrawal was attempted without prior liver histology; prothrombin was found to be predictive of 30-year overall and native liver survivals. The results allow a softer approach to treatment withdrawal in children, avoiding the risks of a liver biopsy, and they provide a tool to help anticipate the need for liver transplantation before complications occur.

Keywords: autoimmune liver disease; liver transplantation; prothrombin ratio; treatment withdrawal.