Ansgar W Lohse1,2, Marcial Sebode1,2, Prithi S Bhathal3, Andrew D Clouston4, Hans P Dienes5, Dhanpat Jain6, Annette S H Gouw7, Maha Guindi8, Sanjay Kakar9, David E Kleiner10, Till Krech11, Carolin Lackner12, Thomas Longerich13, Romil Saxena14, Luigi Terracciano15,16, Kay Washington17, Sören Weidemann11, Stefan G Hübscher18,19, Dina Tiniakos20,21. 1. Department of Medicine, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany. 2. European Reference Network on Hepatological Diseases (ERN RARE-LIVER). 3. Department of Pathology, University of Melbourne, Melbourne, Victoria, Australia. 4. Molecular and Cellular Pathology, The University of Queensland and Envoi Specialist Pathologists, Brisbane, Queensland, Australia. 5. Department of Pathology, Medical University of Vienna, Vienna, Austria. 6. Department of Anatomic Pathology, Yale University School of Medicine, New Haven, Connecticut, USA. 7. Department of Pathology and Medical Biology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. 8. Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, California, USA. 9. Department of Pathology, University of California, San Francisco, California, USA. 10. Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA. 11. Department of Pathology, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany. 12. Institute of Pathology, Medical University of Graz, Graz, Austria. 13. Institute of Pathology, University Hospital Heidelberg, Heidelberg, Germany. 14. Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA. 15. Department of Biomedical Sciences, Humanitas University Pieve Emanuele, Milan, Italy. 16. IRCCS Humanitas Research Hospital, Milan, Italy. 17. Department of Pathology, Vanderbilt University Medical Center, Nashville, Tennessee, USA. 18. Department of Cellular Pathology, Queen Elizabeth Hospital, Birmingham, UK. 19. Institute for Immunology and Immunotherapy, University of Birmingham, Birmingham, UK. 20. Department of Pathology, Aretaieion Hospital, National and Kapodistrian University of Athens, Athens, Greece. 21. Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle, UK.
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.
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.