Rogowska Jagoda, Kubicka Jagoda, Grabowska Martyna, Mirocha Grzegorz, Roszkiewicz Justyna, Smolewska Elżbieta
Department of Pediatric Cardiology and Rheumatology, Medical University of Lodz, Sporna 36/50, 91-738, Lodz, Poland.
Department of Biostatistics and Translational Medicine, Medical University of Lodz, Lodz, Poland.
Clin Rheumatol. 2025 May;44(5):2023-2029. doi: 10.1007/s10067-025-07420-0. Epub 2025 Mar 27.
The study aimed to analyze the clinical and laboratory characteristics of patients with systemic juvenile idiopathic arthritis (sJIA) who developed macrophage activation syndrome (MAS) and identify factors linked to MAS occurrence.
Patients with sJIA, diagnosed based on internationally recognized criteria, participated in the research. Retrospective clinical and laboratory data from inflammatory episodes complicated by MAS were evaluated. The Hemophagocytic Syndrome diagnostic score was calculated to assess its utility on pediatric population.
The study included 23 patients with systemic juvenile idiopathic arthritis (sJIA), of whom 7 (30%) experienced macrophage activation syndrome (MAS) during their disease course. At the time of admission, patients who developed MAS exhibited elevated liver enzymes: ALT (Median = 43, IQR: 25 - 473 vs. Median = 14, IQR: 12 - 30, p = 0.006) and AST (Median = 66, IQR: 51 - 666 vs. Median = 27, IQR: 25 - 33, p = 0.024). Similarly, during hospitalization, patients with MAS showed significantly higher AST levels (Median = 534, IQR: 111 - 1130 vs. Median = 31, IQR: 26 - 42, p = 0.020) and ALT levels (Median = 160, IQR: 46 - 559 vs. Median = 23, IQR: 14 - 48, p = 0.024). Additionally, they experienced a greater decrease in platelet count (Median = 119, IQR: 39 - 209 vs. Median = 306, IQR: 249 - 457, p = 0.004), higher white blood cell counts (Median = 29, IQR: 17 - 36 vs. Median = 18, IQR: 14 - 26, p = 0.021), and elevated D-dimer levels (Median = 16,800, IQR: 5,190 - 34,000 vs. Median = 1,851, IQR: 1,352 - 2,189, p = 0.006). No association was found between MAS and gender, muscle pain, hemophagocytosis in bone marrow aspirate, erythematous rash, or conjunctivitis. The Hemophagocytic Syndrome diagnostic score (HS score) at the optimal cutoff of 169 achieved a sensitivity of 86% and a specificity of 88%.
The findings underscore the significance of recognizing specific clinical and laboratory indicators, such as thrombocytopenia and elevated markers like D-dimers and liver enzymes, to identify sJIA patients at heightened risk for MAS. Prompt diagnosis and intervention are crucial to improving outcomes in this patient group. Key Points • The study identifies thrombocytopenia, elevated D-dimers, hypertriglyceridemia, and elevated liver enzymes (AST, ALT) as key laboratory predictors of macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis (sJIA) patients. • Laboratory abnormalities, including high ferritin levels, thrombocytopenia, and impaired liver function, were highlighted as critical markers for distinguishing MAS from active sJIA. • Clinical factors such as erythematous rash and conjunctivitis were inversely associated with MAS occurrence, aiding differential diagnosis. • The findings emphasize the importance of early detection and routine monitoring of laboratory parameters to prevent delays in MAS diagnosis and improve patient outcome.
本研究旨在分析发生巨噬细胞活化综合征（MAS）的系统性幼年特发性关节炎（sJIA）患者的临床和实验室特征，并确定与MAS发生相关的因素。
根据国际公认标准诊断为sJIA的患者参与了本研究。对并发MAS的炎症发作的回顾性临床和实验室数据进行了评估。计算噬血细胞综合征诊断评分，以评估其在儿科人群中的效用。
该研究纳入了23例系统性幼年特发性关节炎（sJIA）患者，其中7例（30%）在病程中发生了巨噬细胞活化综合征（MAS）。入院时，发生MAS的患者肝酶升高：谷丙转氨酶（ALT）（中位数=43，四分位间距：25 - 473 vs.中位数=14，四分位间距：12 - 30，p = 0.006）和谷草转氨酶（AST）（中位数=66，四分位间距：51 - 666 vs.中位数=27，四分位间距：25 - 33，p = 0.024）。同样，在住院期间，MAS患者的AST水平（中位数=534，四分位间距：111 - 1130 vs.中位数=31，四分位间距：26 - 42，p = 0.020）和ALT水平（中位数=160，四分位间距：46 - 559 vs.中位数=23，四分位间距：14 - 48，p = 0.024）显著更高。此外，他们的血小板计数下降幅度更大（中位数=119，四分位间距：39 - 209 vs.中位数=306，四分位间距：249 - 457，p = 0.004），白细胞计数更高（中位数=29，四分位间距：17 - 36 vs.中位数=18，四分位间距：14 - 26，p = 0.021），D-二聚体水平升高（中位数=16,800，四分位间距：5,190 - 34,000 vs.中位数=1,851，四分位间距：1,352 - 2,189，p = 0.006）。未发现MAS与性别、肌肉疼痛、骨髓穿刺中的噬血细胞现象、红斑皮疹或结膜炎之间存在关联。噬血细胞综合征诊断评分（HS评分）在最佳临界值169时，敏感性为86%，特异性为88%。
研究结果强调了识别特定临床和实验室指标的重要性，如血小板减少以及D-二聚体和肝酶等标志物升高，以识别发生MAS风险较高的sJIA患者。及时诊断和干预对于改善该患者群体的预后至关重要。要点 • 该研究确定血小板减少、D-二聚体升高、高甘油三酯血症和肝酶（AST、ALT）升高是系统性幼年特发性关节炎（sJIA）患者巨噬细胞活化综合征（MAS）的关键实验室预测指标。 • 包括高铁蛋白水平、血小板减少和肝功能受损在内的实验室异常被强调为区分MAS与活动性sJIA的关键标志物。 • 红斑皮疹和结膜炎等临床因素与MAS发生呈负相关，有助于鉴别诊断。 • 研究结果强调了早期检测和常规监测实验室参数对于防止MAS诊断延迟和改善患者预后的重要性。
