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The purpose of this study was to analyze the clinical characteristics and therapeutic effects of AS-AIH.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Retrospective analysis was performed. AIH was diagnosed according to the International Autoimmune Hepatitis Group (IAIHG) criteria revised in 1999. AS-AIH was defined as an acute presentation (onset of symptoms to presentation of \u2264\u200926\u00a0weeks) and INR of \u2265\u20091.5, and no histologic evidence of cirrhosis.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Twelve patients were diagnosed as AS-AIH. At baseline, median immunoglobulin G was 28.35\u00a0g\/L (range, 11.4\u201349.2). Ten (83.3%) patients were antinuclear antibodies and\/or anti-smooth muscle antibodies positive. The prominent histologic characteristics were lobular necrosis\/inflammation (91.7%) and plasma cell infiltration (100%). All patients received corticosteroid therapy. Death occurred in 2 (16.7%) patients within 30\u00a0days resulted from ALF. The average interval between the onset of symptoms and initiation of corticosteroid therapy in deceased patients was 65\u00a0days, compared with 19\u00a0days for survivors.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>AS-AIH is an uncommon disease with poor outcomes. Patients with acute severe hepatitis of unknown cause should be minded the possibility of AS-AIH and corticosteroids should be considered as soon as possible.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12876-021-01653-4","type":"journal-article","created":{"date-parts":[[2021,3,1]],"date-time":"2021-03-01T20:02:58Z","timestamp":1614628978000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Clinical characteristics and treatment outcomes of acute severe autoimmune hepatitis"],"prefix":"10.1186","volume":"21","author":[{"given":"Linhua","family":"Zheng","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yansheng","family":"Liu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yulong","family":"Shang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zheyi","family":"Han","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ying","family":"Han","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,3,1]]},"reference":[{"key":"1653_CR1","doi-asserted-by":"publisher","first-page":"498","DOI":"10.1111\/j.1872-034X.2011.00808.x","volume":"41","author":"H Takahashi","year":"2011","unstructured":"Takahashi H, Zeniya M. 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The procedures followed in this study was approved by the Ethics Committee of Xijing Hospital with the ethical guidelines of the 1975 declaration of Helsinki.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"93"}}