{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,18]],"date-time":"2026-03-18T16:00:21Z","timestamp":1773849621668,"version":"3.50.1"},"reference-count":18,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,1,6]],"date-time":"2024-01-06T00:00:00Z","timestamp":1704499200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Hepatology Research"],"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>A20 haploinsufficiency (HA20) is a recently described autoinflammatory disease that manifests symptoms similar to those of Beh\u00e7et's disease. However, little is known about the involvement of the liver in HA20. Here, we report a case of HA20 complicated by autoimmune hepatitis (AIH).<\/jats:p><\/jats:sec><jats:sec><jats:title>Case presentation<\/jats:title><jats:p>A 33\u2010year\u2010old woman was previously diagnosed with HA20 and chronic thyroiditis, and was treated with prednisolone (PSL; 7.5\u00a0mg\/day) and levothyroxine sodium hydrate (125\u00a0\u03bcg\/day). She experienced general malaise and jaundice, and biochemical evaluation revealed elevated liver function with an aspartate aminotransferase level of 817\u00a0U\/L, an alanine aminotransferase level of 833\u00a0U\/L, and a total bilirubin of 8.3\u00a0mg\/dL. Pathological evaluation of the liver biopsy revealed interface hepatitis and the patient was diagnosed with acute exacerbation of AIH. Upon increasing the PSL dose to 60\u00a0mg\/day, the liver enzyme levels rapidly decreased. During tapering of PSL, azathioprine 50\u00a0mg\/day was added, and there was no relapse of AIH with combination therapy of PSL 7\u00a0mg\/day and azathioprine 50\u00a0mg\/day.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This is the first report of biopsy\u2010proven AIH in an Asian patient with HA20. This case has significant implications for the pathogenesis and treatment of AIH in patients with HA20.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hepr.14003","type":"journal-article","created":{"date-parts":[[2023,12,21]],"date-time":"2023-12-21T11:30:21Z","timestamp":1703158221000},"page":"606-611","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["A case of A20 haploinsufficiency complicated by autoimmune hepatitis"],"prefix":"10.1111","volume":"54","author":[{"given":"Taisei","family":"Iwasa","sequence":"first","affiliation":[{"name":"Department of Gastroenterology\/Internal Medicine Graduate School of Medicine Gifu University  Gifu Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6373-260X","authenticated-orcid":false,"given":"Takao","family":"Miwa","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology\/Internal Medicine Graduate School of 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