{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T01:38:07Z","timestamp":1793151487336,"version":"4.5.0"},"reference-count":33,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,12,5]],"date-time":"2025-12-05T00:00:00Z","timestamp":1764892800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Macrophage activation syndrome (MAS) is among the most life-threatening complications of systemic juvenile idiopathic arthritis (sJIA). Early identification of risk factors is critical for reducing mortality. However, existing evidence is markedly heterogeneous and lacks quantified, comparable, and clinically translatable conclusions.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We systematically searched PubMed, Web of Science, EMBASE, and Chinese databases, including China national knowledge infrastructure (CNKI), Wanfang, VIP, and Chinese biomedical literature database (CBM), from inception to 1 July 2025, for case\u2013control studies investigating risk factors for MAS in sJIA. Two investigators independently screened studies, extracted data, and assessed study quality. Meta-analyses were performed using RevMan 5.3 and Stata 16.0.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Result<\/jats:title>\n                    <jats:p>A total of 10 studies involving 1,936 patients were included, comprising 643 cases and 1,293 controls. Meta-analysis showed that central nervous system (CNS) involvement, hypofibrinogenemia, markedly elevated ferritin levels, and leukopenia were significantly associated with MAS in sJIA, with odds ratios (95% confidence intervals) of 4.30 (2.13\u20138.65), 4.03 (2.87\u20135.65), 8.28 (5.66\u201312.10), and 5.98 (2.80\u201312.75), respectively.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>CNS involvement, hypofibrinogenemia, hyperferritinemia, and leukopenia are core risk factors for MAS in patients with sJIA.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Systematic Review Registration<\/jats:title>\n                    <jats:p>PROSPERO CRD420251108082.<\/jats:p>\n                  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Rheumatol"},{"key":"B11","doi-asserted-by":"publisher","first-page":"203","DOI":"10.1016\/j.jpeds.2021.02.008","article-title":"Sudden hypotension and increased Serum interferon-\u03b3 and interleukin-10 predict early macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis","volume":"235","author":"Guo","year":"2021","journal-title":"J Pediatr"},{"key":"B12","doi-asserted-by":"publisher","first-page":"206","DOI":"10.3390\/jcm11010206","article-title":"Detection and prediction of macrophage activation syndrome in Still\u2019s disease","volume":"11","author":"Javaux","year":"2021","journal-title":"J Clin Med"},{"key":"B13","doi-asserted-by":"publisher","first-page":"417","DOI":"10.1016\/j.semarthrit.2014.09.004","article-title":"Identification of the best cutoff points and clinical signs specific for early recognition of macrophage activation syndrome in active systemic juvenile idiopathic arthritis","volume":"44","author":"Kostik","year":"2015","journal-title":"Semin Arthritis Rheum"},{"key":"B14","unstructured":"Shejie\n              L\n            \n          \n          Capital Medical University\n          Study on early warning indicators of systemic juvenile idiopathic arthritis complicated with MAS\n          \n          2017"},{"key":"B15","first-page":"37","article-title":"Research on the establishment of an early warning scale for macrophage activation syndrome in children with systemic juvenile idiopathic arthritis","volume":"37","author":"Xiaohua","year":"2022","journal-title":"J Chinese J Pract Pediatr"},{"key":"B16","unstructured":"Wenxiu\n              X\n            \n          \n          Suzhou University\n          Clinical analysis of 45 cases of systemic juvenile idiopathic arthritis complicated with macrophage activation syndrome\n          \n          2023"},{"key":"B17","unstructured":"Tingting\n              Z\n            \n          \n         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