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Consensus recommendations for histological criteria of autoimmune hepatitis from the International AIH Pathology Group: Results of a workshop on AIH histology hosted by the European Reference Network on Hepatological Diseases and the European Society of Pathology
Abstract
Background & Aims: Diagnostic histological criteria for autoimmune hepatitis (AIH) have not been clearly established. Previously published criteria focused mainly on chronic AIH, in which inflammatory changes mainly occur in portal/periportal regions and may not be applicable to acute presentation of AIH, in which inflammatory changes are typically predominantly lobular in location. International consensus criteria for the diagnosis and assessment of disease severity in both acute and chronic AIH are thus urgently needed. Methods: Seventeen expert liver pathologists convened at an international workshop and subsequently used a modified Delphi panel approach to establish consensus criteria for the histopathological diagnosis of AIH. Results: The consensus view is that liver biopsy should remain standard for diagnosing AIH. AIH is considered likely, if there is a predominantly portal lymphoplasmacytic hepatitis with more than mild interface activity and/or more than mild lobular hepatitis in the absence of histological features suggestive of another liver disease. AIH is also considered likely if there is predominantly lobular hepatitis with or without centrilobular necroinflammation and at least one of the following features: portal lymphoplasmacytic hepatitis, interface hepatitis or portal‐based fibrosis, in the absence of histological features suggestive of another liver disease. Emperipolesis and hepatocellular rosettes are not regarded as being specific for AIH. Conclusions: The criteria proposed in this consensus statement provide a uniform approach to the histological diagnosis of AIH, which is relevant for patients with an acute as well as a chronic presentation and to more accurately reflect the current understanding of liver pathology in AIH.
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Histology showed lobular hepatitis (76%) and portal hepatitis (17%), with fibrosis being more prominent in the latter, which favoured the diagnosis of DI-ALH rather than AIH, despite the fact that 82% of patients had typical or probable AIH on simplified IAIHG scoring and that 92% of patients had likely or possible AIH based on the ERN histology system.
“Histology showed lobular hepatitis (76%) and portal hepatitis (17%), with fibrosis being more prominent in the latter, which favoured the diagnosis of DI-ALH rather than AIH, despite the fact that 82% of patients had typical or probable AIH on simplified IAIHG scoring46 and that 92% of patients had likely or possible AIH based on the ERN histology system.”
From Nomenclature, diagnosis and management of drug-induced autoimmune-like hepatitis (DI-ALH): An expert opinion meeting report (2023) · cites this paper as [3]
ALH events are characterised by histological features highly overlapping with idiopathic AIH and often associated with serum liver autoantibodies and elevated IgG levels.
“ALH events are characterised by histological features highly overlapping with ‘idiopathic’ (classical) AIH and often associated with the presence of serum liver autoantibodies and elevated IgG levels.”
From Nomenclature, diagnosis and management of drug-induced autoimmune-like hepatitis (DI-ALH): An expert opinion meeting report (2023) · cites this paper as [3]
The specificity of emperipolesis and rosette formation for AIH has been questioned and might reflect disease severity rather than aetiology of liver injury.
“The specificity of emperipolesis and rosette formation for AIH has been questioned and might reflect disease severity rather than aetiology of liver injury.”
From Nomenclature, diagnosis and management of drug-induced autoimmune-like hepatitis (DI-ALH): An expert opinion meeting report (2023) · cites this paper as [3]
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EASL Clinical Practice Guidelines: autoimmune hepatitis
(2015) · Journal of Hepatology · doi:10.1016/j.jhep.2015.06.030 · Cited by 1,245
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Andrade, R. J., Aithal, G. P., de Boer, Y. S., Liberal, R., Gerbes, A., Regev, A., Terziroli Beretta-Piccoli, B., Schramm, C., Kleiner, D. E., De Martin, E., et al. (2023) · Journal of Hepatology · doi:10.1016/j.jhep.2023.04.033 · Cited by 166
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Metadata
- Authors
- Lohse, A. W., Sebode, M., Bhathal, P. S., Clouston, A. D., Dienes, H. P., Jain, D., Gouw, A. S. H., Guindi, M., Kakar, S., Kleiner, D. E., Krech, T., Lackner, C., Longerich, T., Saxena, R., Terracciano, L., Washington, K., Weidemann, S., Hübscher, S. G., & Tiniakos, D.
- Venue
- Liver International
- Publisher
- Wiley
- Published
- 2022-03-12
- Volume
- 42
- Issue
- 5
- Pages
- 1058-1069
- ISSN
- 1478-3223
- 1478-3231
- Cited by
- 153
- References
- 1
- Language
- en
- Funders
- European Society for Photobiology
- European Commission
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