{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T01:01:41Z","timestamp":1792803701131,"version":"4.5.0"},"reference-count":84,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2024,6,21]],"date-time":"2024-06-21T00:00:00Z","timestamp":1718928000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Institute of Clinical Medicine Research of Bucheon St. Mary\u2019s Hospital, Research Fund, 2024","id":[{"id":"https:\/\/ror.org\/0443jbw36","id-type":"ROR","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Macrophage activation syndrome (MAS) is potentially fatal; so, early diagnosis and timely treatment are essential. However, detecting MAS is sometimes challenging because its principal features can be observed in other pediatric diseases that cause severe inflammation. Cytokine storm due to immune dysregulation represents the clinical and laboratory features of MAS that are included in the diagnostic criteria. Most cases of MAS occur as an underlying condition worsens and progresses. Therefore, a patient with autoimmune or autoinflammatory disease who shows unexplained clinical deterioration despite appropriate management should be considered at high risk for MAS (i.e., occult MAS). The basic principles of treatment are control of triggering factors, supportive care, and relief of hyperinflammation. Systemic steroids and cyclosporine A are frequently used as a first-line treatment. For the treatment of refractory MAS, cytokine-specific biologic agents such as anakinra have recently become preferred over traditional immunosuppressive agents such as etoposide. MAS might be underrecognized in pediatric patients with infectious and inflammatory diseases due to its diverse clinical presentations. Clinical suspicion of MAS is of the utmost importance for early recognition of the disease.<\/jats:p>","DOI":"10.3390\/children11070755","type":"journal-article","created":{"date-parts":[[2024,6,21]],"date-time":"2024-06-21T08:50:08Z","timestamp":1718959808000},"page":"755","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Macrophage Activation Syndrome in Children: Update on Diagnosis and Treatment"],"prefix":"10.3390","volume":"11","author":[{"given":"Jin","family":"Lee","sequence":"first","affiliation":[{"name":"Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul 06591, Republic of Korea"},{"name":"Department of Pediatrics, Incheon St. Mary\u2019s Hospital, The Catholic University of Korea, Incheon 21431, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kil Seong","family":"Bae","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul 06591, Republic of Korea"},{"name":"Department of Pediatrics, Eunpyeong St. Mary\u2019s Hospital, The Catholic University of Korea, Seoul 03312, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jung Woo","family":"Rhim","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul 06591, Republic of Korea"},{"name":"Department of Pediatrics, Daejeon St. Mary\u2019s Hospital, The Catholic University of Korea, Daejeon 34943, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5354-3135","authenticated-orcid":false,"given":"Soo-Young","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul 06591, Republic of Korea"},{"name":"Department of Pediatrics, Bucheon St. Mary\u2019s Hospital, The Catholic University of Korea, Bucheon 14647, Republic of Korea"},{"name":"The Vaccine Bio Research Institute, College of Medicine, The Catholic University of Korea, Seoul 06591, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dae Chul","family":"Jeong","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul 06591, Republic of Korea"},{"name":"The Vaccine Bio Research Institute, College of Medicine, The Catholic University of Korea, Seoul 06591, Republic of Korea"},{"name":"Department of Pediatrics, Seoul St. Mary\u2019s Hospital, The Catholic University of Korea, Seoul 06591, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jin Han","family":"Kang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul 06591, Republic of Korea"},{"name":"The Vaccine Bio Research Institute, College of Medicine, The Catholic University of Korea, Seoul 06591, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,6,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"927","DOI":"10.1016\/j.hoc.2015.06.010","article-title":"Macrophage activation syndrome","volume":"29","author":"Ravelli","year":"2015","journal-title":"Hematol. 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