Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, 06100, Ankara, Turkey.
Faculty of Medicine, Hacettepe University, Ankara, Turkey.
Clin Rheumatol. 2024 Jan;43(1):415-421. doi: 10.1007/s10067-023-06828-w. Epub 2023 Nov 28.
Macrophage activation syndrome (MAS) is a severe complication of systemic juvenile idiopathic arthritis (SJIA). We aimed to compare the characteristics of SJIA patients who developed MAS in the disease course to those who never experienced MAS.
Patients with SJIA were included. The features of the patients at the time of SJIA diagnosis were compared. Multivariate logistic regression and ROC analyses were used while evaluating factors associated with MAS.
Overall, 126 SJIA patients (M/F:1.17) were included. Eighty-six (68.2%) never had MAS. At the time of SJIA diagnosis, age was younger; the duration of fever was longer; rash, hepatomegaly, and splenomegaly were more frequent and arthralgia/arthritis was less common among patients who had MAS in the follow-up than those who never had MAS. Also, white blood cell, neutrophil, and platelet counts and fibrinogen were lower, while transaminases, lactate dehydrogenase, triglyceride (TG), and ferritin levels were higher among patients with MAS than those without MAS. The multivariate regression analysis disclosed age at symptom onset, duration of fever, platelet count, TG and ferritin levels as independent MAS predictors. For ferritin level/platelet count (F/P) ratio at the time of SJIA diagnosis, a threshold of ≥1.1 performed best to predict a MAS-prone disease course with a sensitivity of 90% and a specificity of 82.6%.
The F/P ratio at the time of SJIA diagnosis may be a promising biomarker to predict MAS-prone disease course in SJIA. Determining MAS-prone patients at the time of SJIA diagnosis could assist physicians while tailoring SJIA treatment individually. Key points • Systemic juvenile idiopathic arthritis (SJIA) patients with macrophage activation syndrome (MAS) differ from SJIA patients who never have MAS, at the time of SJIA diagnosis. • It could be possible to predict a MAS-prone disease course at the time of SJIA diagnosis. • The ferritin/platelet ratio is a promising biomarker for predicting MAS-prone SJIA disease course.
巨噬细胞活化综合征（MAS）是全身型幼年特发性关节炎（SJIA）的一种严重并发症。本研究旨在比较 SJIA 患者在病程中发生 MAS 与从未发生 MAS 的患者的特征。
纳入 SJIA 患者。比较患者 SJIA 诊断时的特征。采用多变量逻辑回归和 ROC 分析评估与 MAS 相关的因素。
共纳入 126 例 SJIA 患者（男/女：1.17）。86 例（68.2%）从未发生 MAS。在 SJIA 诊断时，年龄更小；发热持续时间更长；皮疹、肝脾肿大更常见，关节炎/关节痛更少见。MAS 患者的白细胞、中性粒细胞、血小板和纤维蛋白原计数较低，而转氨酶、乳酸脱氢酶、甘油三酯（TG）和铁蛋白水平较高。多变量回归分析显示，发病年龄、发热持续时间、血小板计数、TG 和铁蛋白水平是 MAS 的独立预测因素。在 SJIA 诊断时，铁蛋白/血小板计数（F/P）比值的临界值≥1.1 可最佳预测 MAS 倾向疾病过程，其敏感性为 90%，特异性为 82.6%。
SJIA 诊断时的 F/P 比值可能是预测 SJIA 易发生 MAS 疾病过程的有前途的生物标志物。在 SJIA 诊断时确定易发生 MAS 的患者可以帮助医生为每位患者量身定制 SJIA 治疗方案。
全身型幼年特发性关节炎（SJIA）伴巨噬细胞活化综合征（MAS）患者与从未发生 MAS 的 SJIA 患者在 SJIA 诊断时存在差异。
有可能在 SJIA 诊断时预测 MAS 倾向的疾病过程。
铁蛋白/血小板比值是预测 MAS 倾向 SJIA 疾病过程的有前途的生物标志物。
