@article{01J6Y1KY46SMD1FXGDK31JBTKN,
  abstract     = {{Background & Aims: The goal of treatment in autoimmune hepatitis (AIH) is induction of remission to prevent the development of liver fibrosis, cirrhosis, and its related complications. Various definitions of treatment response and remission have been used. The International Autoimmune Hepatitis Group (IAIHG) recently defined consensus criteria for treatment response. We aimed to validate the IAIHG response criteria in our cohort and establish correlations with survival endpoints. Methods: We performed a retrospective, multicentric cohort study in one tertiary and seven secondary care centres in Belgium. Eligible patients were at least 18 years of age at data collection and were diagnosed with AIH by a simplified IAIHG score of >= 6. Complete biochemical response (CBR) was defined according to the IAIHG consensus criteria as normalisation of transaminases and serum IgG within the first 6 months of treatment. The primary endpoint was liver-related survival - defined as freedom from liver-related death or liver transplantation. Secondary endpoints were overall mortality and transplant-free survival. Outcomes were compared between patients attaining CBR and those with insufficient response. Results: Biochemical response status could be determined in 200 patients with AIH: CBR was achieved in 128 (64.0%) individuals. Patients not achieving CBR more frequently presented with cirrhosis on initial histology (22.2% vs. 10.9%, p = 0.036). Liver-related mortality or liver transplantation as a primary outcome occurred in 26 patients (13.0%). Patients achieving CBR exhibited superior liver-related (hazard ratio 0.118; 95% CI 0.052-0.267; p <0.0001) and overall (hazard ratio 0.253; 95% CI 0.111-0.572; p = 0.0003) survival. Conclusions: We externally validated the IAIHG consensus criteria for CBR and confirmed their correlation with survival endpoints in a multicentric, real-world cohort. Patients with AIH achieving CBR as an intermediate endpoint have significantly superior liver-related and overall survival. (c) 2024 The Authors. Published by Elsevier B.V. on behalf of European Association for the Study of the Liver (EASL). This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).}},
  articleno    = {{101149}},
  author       = {{Grossar, Lorenz and Raevens, Sarah and Van Steenkiste, Christophe and Colle, Isabelle and De Vloo, Charlotte and  Orlent, Hans and Schouten, Jeoffrey and  Gallant, Marie and  Van Driessche, Annelien and Lefere, Sander and Devisscher, Lindsey and Geerts, Anja and Van Vlierberghe, Hans and Verhelst, Xavier}},
  issn         = {{2589-5559}},
  journal      = {{JHEP REPORTS}},
  keywords     = {{CONTROLLED-TRIAL,NATURAL-HISTORY,LIVER-DISEASE,THERAPY,BUDESONIDE,PHARMACOKINETICS,PREDNISONE,REMISSION,PROGNOSIS,DIAGNOSIS}},
  language     = {{eng}},
  number       = {{9}},
  pages        = {{8}},
  title        = {{External validation of the IAIHG autoimmune hepatitis response criteria in a multicentric real-world cohort}},
  url          = {{http://doi.org/10.1016/j.jhepr.2024.101149}},
  volume       = {{6}},
  year         = {{2024}},
}

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