Terziroli Beretta-Piccoli, Benedetta Mieli Vergani, Giorgina Vergani, Diego 2021-09-27 Autoimmune hepatitis (AIH) is a T-cell mediated, inflammatory liver disease affecting all ages and characterized by female preponderance, elevated serum transaminase and immunoglobulin G levels, positive circulating autoantibodies, and presence of interface hepatitis at liver histology. AIH type 1, affecting both adults and children, is defined by positive anti- nuclear and/or antismooth muscle antibodies, while type 2 AIH, affecting mostly children, is defined by positive anti-liver- kidney microsomal type 1 and/or anti-liver cytosol type 1 antibody. While the autoantigens of type 2 AIH are well defined, being the cytochrome P4502D6 (CYP2D6) and the formiminotransferase cyclodeaminase (FTCD), in type 1 AIH they remain to be identified. AIH-1 predisposition is conferred by possession of the MHC class II HLA DRB1*03 at all ages, while DRB1*04 predisposes to late onset disease; AIH-2 is associated with possession of DRB1*07 and DRB1*03. The majority of patients responds well to standard immunosuppressive treatment, based on steroid and azathioprine; second- and third-line drugs should be considered in case of intolerance or insufficient response. This review offers a comprehensive overview of pathophysiological and clinical aspects of AIH. application/pdf https://susi.usi.ch/global/documents/319292 https://localhost:5000/ark:/12658/srd1319292 https://susi.usi.ch/documents/319292/files/TerziroliBeretta-Piccoli_cmi_2021.pdf eng info:eu-repo/semantics/altIdentifier/doi/10.1038/s41423-021-00768-8 info:eu-repo/semantics/altIdentifier/ark/12658/srd1319292 info:eu-repo/semantics/openAccess CC BY Cellular & molecular immunology. - Springer. - 2022, vol. 19, p. 158–176 Autoimmune Hepatitis Immunopathophysiology Treatment Genetic Predisposition info:eu-repo/classification/udc/61 Autoimmmune hepatitis http://purl.org/coar/resource_type/c_6501