SYSTEMATIC REVIEW article
Front. Pediatr.
Sec. Pediatric Rheumatology
Risk Factors for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis: A Systematic Review and Meta-Analysis
Provisionally accepted
- Sichuan University West China Second University Hospital, Chengdu, China
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Background 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. Methods We systematically searched PubMed, Web of Science, EMBASE, and Chinese databases including CNKI, Wanfang, VIP, and CBM from inception to July 1, 2025, for case–control 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. Result 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 (2.13–8.65), 4.03 (2.87–5.65), 8.28 (5.66–12.10), and 5.98 (2.80 –12.75), respectively. Conclusion CNS involvement, hypofibrinogenemia, hyperferritinemia, and leukopenia are core risk factors for MAS in patients with sJIA.
Keywords: Systemic juvenile idiopathic arthritis, Macrophage Activation Syndrome, Risk factors, Case–control study, Meta-analysis
Received: 30 Aug 2025; Accepted: 20 Nov 2025.
Copyright: © 2025 Li, Wu, Jing, Yang, Zhang and Dong. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Liqun Dong, dongliqun@scu.edu.cn
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