Liu Yingying, Pan Yuting, Jin Jing, Wang Panpan, Zhang Tonghao, Fan Zhidan, Yu Haiguo
Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, China.
Front Pediatr. 2024 Dec 17;12:1469912. doi: 10.3389/fped.2024.1469912. eCollection 2024.
This study aimed to assess the diagnosis of macrophage activation syndrome (MAS) at the onset of active childhood-onset systemic lupus erythematosus (cSLE), which is under-researched, and to compare the characteristics of cSLE with and without MAS, hypothesizing the existence of possible predictors of MAS in active cSLE.
This study enrolled 157 patients diagnosed with cSLE, with or without MAS, from Nanjing Medical University between January 2018 and May 2023. Data analysis was performed using an independent samples -test or the Mann-Whitney -test, the test, the Youden index to determine the optimal cutoff values for diagnosis, and binary logistic regression analysis to determine the predicted probability.
Fifteen patients (9%) had MAS in the active phase, with an SLE disease activity index of 16.6 (range, 6-32). Bone marrow aspirations revealed hemophagocytosis in 8/15 cases (53%). Fever was the most common feature of MAS patients. Lactate dehydrogenase (LDH) and ferritin levels were elevated in the patients. Lower leukocyte, neutrophil, and platelet counts, including serum sodium and fibrinogen, and increased alanine aminotransferase, aspartate aminotransferase (AST), lactate dehydrogenase (LDH), ferritin, triglyceride, and D-dimer levels occurred in MAS patients, unlike those without MAS. Optimal cutoff values for ferritin (≥607.35 ng/ml), LDH (≥424 U/L), and AST (≥61 U/L) were predictors of MAS occurrence in cSLE. No MAS patients experienced recurrence during an 18-month mean follow-up.
Despite the narrow scope of the study, elevated levels of ferritin, LDH, and AST may represent indicators of cSLE complicated by MAS. Early diagnosis and treatment may improve outcomes.
本研究旨在评估儿童期起病的系统性红斑狼疮（cSLE）活动期巨噬细胞活化综合征（MAS）的诊断情况，目前该方面研究较少，并比较有和无MAS的cSLE的特征，推测活动期cSLE中可能存在MAS的预测指标。
本研究纳入了2018年1月至2023年5月期间在南京医科大学诊断为cSLE的157例患者，这些患者有或无MAS。使用独立样本t检验或曼-惠特尼U检验、卡方检验、约登指数来确定诊断的最佳临界值，并进行二元逻辑回归分析以确定预测概率。
15例患者（9%）在活动期患有MAS，SLE疾病活动指数为16.6（范围6 - 32）。骨髓穿刺显示15例中有8例（53%）存在噬血细胞现象。发热是MAS患者最常见的特征。患者乳酸脱氢酶（LDH）和铁蛋白水平升高。与无MAS的患者不同，MAS患者白细胞、中性粒细胞和血小板计数降低，包括血清钠和纤维蛋白原水平降低，而丙氨酸氨基转移酶、天冬氨酸氨基转移酶（AST）、乳酸脱氢酶（LDH）、铁蛋白、甘油三酯和D - 二聚体水平升高。铁蛋白（≥607.35 ng/ml）、LDH（≥424 U/L）和AST（≥61 U/L）的最佳临界值是cSLE中MAS发生的预测指标。在平均18个月的随访期间，无MAS患者复发。
尽管本研究范围有限，但铁蛋白、LDH和AST水平升高可能是cSLE合并MAS的指标。早期诊断和治疗可能改善预后。
