Department of Rheumatology Immunology & Allergy, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Binsheng Rd 3333, Binjiang District, Hangzhou, 310052, PR China.
Pediatr Rheumatol Online J. 2023 Oct 12;21(1):115. doi: 10.1186/s12969-023-00907-7.
Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases, predominantly in systemic juvenile idiopathic arthritis (SJIA), and is considered as an autoinflammatory disease. Specific cytokine profiles could play a pivotal role in this inflammatory response. Gram-negative bacteremia, bacterial pneumonia, Kawasaki disease, and active SJIA exhibited similar cytokine profiles with elevated interleukin-6 (IL-6) and/or IL-10, further suggesting a correlation between them. Only when JIA is complicated by MAS can increased interferon-γ (IFN-γ) levels be observed. Therefore, increased serum IFN-γ levels could contribute to early diagnosing MAS in patients with SJIA in combination with other variables such as serum ferritin. A prospective multi-center study will be performed to further confirm the role of IFN-γ in the early recognition of MAS in SJIA.
巨噬细胞活化综合征（MAS）是风湿性疾病的一种严重的、潜在致命的并发症，主要发生在全身型幼年特发性关节炎（SJIA）中，被认为是一种自身炎症性疾病。特定的细胞因子谱可能在这种炎症反应中发挥关键作用。革兰氏阴性菌血症、细菌性肺炎、川崎病和活动期 SJIA 表现出相似的细胞因子谱，白细胞介素-6（IL-6）和/或 IL-10 升高，进一步提示它们之间存在相关性。只有当 JIA 并发 MAS 时，才能观察到干扰素-γ（IFN-γ）水平的升高。因此，血清 IFN-γ水平的升高可能有助于结合其他变量（如血清铁蛋白）早期诊断 SJIA 患者的 MAS。一项前瞻性多中心研究将进一步证实 IFN-γ在 SJIA 中早期识别 MAS 中的作用。
