Department of Rheumatology Immunology & Allergy, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China.
Department of Pediatrics, George Washington University, Washington, DC.
J Pediatr. 2021 Aug;235:203-211.e3. doi: 10.1016/j.jpeds.2021.02.008. Epub 2021 Feb 11.
To identify clinical and laboratory predictors for early macrophage activation syndrome (MAS) associated with systemic juvenile idiopathic arthritis (sJIA).
This is a retrospective cohort study of 149 patients with sJIA, of whom 27 had 31 episodes of MAS. We evaluated the clinical and laboratory features of patients with sJIA and MAS and compared them with those without MAS. We focused our analysis on the overall process of MAS development, especially MAS onset.
As shown in previous studies, we found a high percentage of fever, absence of arthritis, and central nervous system dysfunction at MAS onset in our study cohort. We also found that 35% of patients with MAS had hypotension although not shock, and 22.6% of patients with MAS had gastrointestinal involvement at MAS onset. Compared with patients with MAS without hypotension, patients with MAS and hypotension had greater rates of admission to the intensive care unit; presented with more arthritis, serositis, pneumonia, and gastrointestinal involvement; and had greater white blood cell and absolute neutrophil counts and serum bilirubin levels and lower serum total protein. We confirmed laboratory markers such as platelet counts, lactate dehydrogenase, and aspartate aminotransferase can help to identify early MAS and that ferritin/erythrocyte sedimentation rate ratio of approximately 20.0 had a high diagnostic sensitivity and specificity for MAS. In addition, we discovered that the combination of interferon-γ >17.1 pg/mL and interleukin-10 >7.8 pg/mL appeared to be a good cytokine pattern for the recognition of MAS onset.
Sudden hypotension, elevated ferritin/erythrocyte sedimentation rate ratio, and the cytokine pattern of significantly increased interferon-γ and interleukin-10 levels are important markers for early identification of MAS in addition to the traditional characteristics of sJIA-associated MAS.
确定与全身幼年特发性关节炎（sJIA）相关的早期巨噬细胞活化综合征（MAS）的临床和实验室预测因素。
这是一项对 149 例 sJIA 患者的回顾性队列研究，其中 27 例患者出现 31 次 MAS 发作。我们评估了 sJIA 和 MAS 患者的临床和实验室特征，并将其与无 MAS 的患者进行了比较。我们的分析重点是 MAS 发展的整个过程，尤其是 MAS 的发作。
正如之前的研究所示，我们在研究队列中发现 MAS 发作时高热、关节炎缺失和中枢神经系统功能障碍的比例较高。我们还发现，尽管没有休克，但 35%的 MAS 患者存在低血压，22.6%的 MAS 患者在 MAS 发作时存在胃肠道受累。与无低血压的 MAS 患者相比，患有 MAS 和低血压的患者入住重症监护病房的比例更高；表现出更多的关节炎、胸膜炎、肺炎和胃肠道受累；白细胞和绝对中性粒细胞计数、血清胆红素水平更低，血清总蛋白水平更高。我们证实了血小板计数、乳酸脱氢酶和天冬氨酸转氨酶等实验室标志物有助于早期识别 MAS，铁蛋白/红细胞沉降率比值约为 20.0 对 MAS 具有较高的诊断敏感性和特异性。此外，我们发现干扰素-γ＞17.1 pg/mL 和白细胞介素-10＞7.8 pg/mL 的组合似乎是识别 MAS 发作的良好细胞因子模式。
除了 sJIA 相关 MAS 的传统特征外，突然出现低血压、铁蛋白/红细胞沉降率比值升高以及干扰素-γ和白细胞介素-10 水平显著升高的细胞因子模式是早期识别 MAS 的重要标志物。
