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MAS is characterized by a dysfunctional immune response that is similar to that seen in other forms of HLH. Because MAS may pursue a rapidly fatal course, prompt recognition of its clinical and laboratory features and immediate therapeutic intervention are fundamental. Recently, a set of classification criteria for MAS complicating sJIA has been developed through a multinational collaborative effort. High\u2010dose parenteral corticosteroids remain the mainstay of treatment of MAS.<\/jats:p>","DOI":"10.1111\/pai.13158","type":"journal-article","created":{"date-parts":[[2020,2,3]],"date-time":"2020-02-03T23:46:51Z","timestamp":1580773611000},"page":"13-15","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Macrophage activation syndrome in pediatrics"],"prefix":"10.1111","volume":"31","author":[{"given":"Alessandra","family":"Alongi","sequence":"first","affiliation":[{"name":"Universit\u00e0 degli Studi di Genova Genoa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberta","family":"Naddei","sequence":"additional","affiliation":[{"name":"Universit\u00e0 degli Studi di Napoli Federico II Napoli Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"De Miglio","sequence":"additional","affiliation":[{"name":"Universit\u00e0 degli Studi del Piemonte Orientale Novara Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Natoli","sequence":"additional","affiliation":[{"name":"Universit\u00e0 degli Studi di Genova Genoa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9658-0385","authenticated-orcid":false,"given":"Angelo","family":"Ravelli","sequence":"additional","affiliation":[{"name":"Universit\u00e0 degli Studi di Genova Genoa Italy"},{"name":"IRCCS Istituto Giannina Gaslini Genoa Italy"},{"name":"Sechenov First Moscow State Medical University Moscow Russian Federation"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,2,3]]},"reference":[{"key":"e_1_2_3_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/gene.2012.3"},{"key":"e_1_2_3_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrrheum.2015.179"},{"key":"e_1_2_3_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.hoc.2015.06.010"},{"key":"e_1_2_3_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.38802"},{"key":"e_1_2_3_6_1","doi-asserted-by":"publisher","DOI":"10.1586\/1744666X.2015.1058159"},{"key":"e_1_2_3_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21039"},{"key":"e_1_2_3_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.38769"},{"key":"e_1_2_3_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2004.12.016"},{"key":"e_1_2_3_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2015-208982"},{"key":"e_1_2_3_11_1","doi-asserted-by":"publisher","DOI":"10.1136\/rmdopen-2015-000161"}],"container-title":["Pediatric Allergy and Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fpai.13158","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pai.13158","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/pai.13158","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pai.13158","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,4]],"date-time":"2023-09-04T21:01:39Z","timestamp":1693861299000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pai.13158"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,2]]},"references-count":10,"journal-issue":{"issue":"S24","published-print":{"date-parts":[[2020,2]]}},"alternative-id":["10.1111\/pai.13158"],"URL":"https:\/\/doi.org\/10.1111\/pai.13158","archive":["Portico"],"relation":{},"ISSN":["0905-6157","1399-3038"],"issn-type":[{"value":"0905-6157","type":"print"},{"value":"1399-3038","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,2]]},"assertion":[{"value":"2019-10-18","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-10-24","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-02-03","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}