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The clinical presentation is often that of acute hepatitis, sometimes very severe; less frequently, it can be insidious or completely asymptomatic. The disease can affect people of any age and is more common in women; its incidence and prevalence seem to be on the rise worldwide. An abnormal immune response targeting liver autoantigens and inducing persistent and self-perpetuating liver inflammation is the pathogenic mechanism of the disease. A specific set of autoantibodies, increased IgG concentrations, and histological demonstration of interface hepatitis and periportal necrosis are the diagnostic hallmarks of autoimmune hepatitis. Prompt response to treatment with corticosteroids and other immunomodulatory drugs is almost universal and supports the diagnosis. The aims of treatment are to induce and maintain long term remission of liver inflammation. Treatment can often even reverse liver fibrosis, thus preventing progression to advanced cirrhosis and its complications. Most patients need lifelong maintenance therapy, and repeated follow-up in experienced hands improves the quality of care and quality of life for affected patients.<\/jats:p>","DOI":"10.1136\/bmj-2022-070201","type":"journal-article","created":{"date-parts":[[2023,2,6]],"date-time":"2023-02-06T06:51:14Z","timestamp":1675666274000},"page":"e070201","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":200,"title":["Diagnosis and management of autoimmune hepatitis"],"prefix":"10.1136","volume":"380","author":[{"given":"Luigi","family":"Muratori","sequence":"first","affiliation":[{"name":"DIMEC, Universit\u00e0 di Bologna and IRCCS Policlinico di Sant\u2019Orsola, Bologna, Italy"},{"name":"European Reference Network for Hepatological Diseases (ERN RARE-LIVER)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ansgar W","family":"Lohse","sequence":"additional","affiliation":[{"name":"Department of Medicine, University 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