Macrophage activation syndrome in systemic juvenile idiopathic arthritis.

IF 2.7 Q3 IMMUNOLOGY Immunological Medicine Pub Date : 2021-12-01 Epub Date: 2021-05-13 DOI:10.1080/25785826.2021.1912893
Masaki Shimizu
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引用次数: 15

Abstract

Macrophage activation syndrome (MAS) is a severe, potentially life-threatening complication of systemic juvenile idiopathic arthritis (s-JIA). An immunological feature is the excessive activation and proliferation of T lymphocytes and macrophages. Massive hypercytokinemia is strongly associated with its pathogenesis, particularly the overproduction of interleukin (IL)-1, IL-6 and IL-18; interferon (IFN)-γ; and tumor necrosis factor (TNF)-α. Furthermore, heterozygous mutations in causative genes for primary hemophagocytic lymphohistiocytosis and in vivo exposure to highly elevated levels of IL-6 and IL-18 might induce natural killer cell dysfunction and decrease their numbers, respectively. A proper diagnosis is important to begin appropriate therapeutic interventions and change an unfavorable prognosis. The 2016 ACR/EULAR classification criteria for MAS have a high diagnostic performance; however, the diagnostic sensitivity for onset is relatively low. Therefore, careful monitoring of laboratory values during the course of MAS is necessary to diagnose it early in s-JIA. Further studies on the diagnosis and monitoring of disease activity using serum cytokine profile and a targeted cytokine strategy are required.

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系统性幼年特发性关节炎的巨噬细胞激活综合征。
巨噬细胞激活综合征(MAS)是系统性青少年特发性关节炎(s-JIA)的一种严重的、可能危及生命的并发症。一个免疫学特征是T淋巴细胞和巨噬细胞的过度激活和增殖。大量高细胞素血症与其发病机制密切相关,特别是白细胞介素(IL)-1、IL-6和IL-18的过量产生;干扰素(IFN) -γ;肿瘤坏死因子(TNF)-α。此外,原发性噬血细胞淋巴组织细胞病致病基因的杂合突变以及体内暴露于高水平的IL-6和IL-18可能分别诱导自然杀伤细胞功能障碍并减少其数量。正确的诊断对于开始适当的治疗干预和改变不良预后非常重要。2016年ACR/EULAR对MAS的分类标准具有较高的诊断性能;然而,对发病的诊断敏感性相对较低。因此,在MAS过程中仔细监测实验室值对于s-JIA的早期诊断是必要的。需要进一步研究使用血清细胞因子谱和靶向细胞因子策略来诊断和监测疾病活动。
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来源期刊
Immunological Medicine
Immunological Medicine Medicine-Immunology and Allergy
CiteScore
7.10
自引率
2.30%
发文量
19
审稿时长
19 weeks
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