Macrophage activation syndrome and cytokine-directed therapies.
Macrophage activation syndrome and cytokine-directed therapies.
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DOI：
10.1016/j.berh.2014.03.002
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发表时间：
2014-04
期刊：
影响因子：
--
通讯作者：
Grom AA
中科院分区：
文献类型：
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作者：
Schulert GS;Grom AA
巨噬细胞活化综合征（MAS）是一种最常见于全身性幼年特发性关节炎儿童的压倒性炎症发作。其特征是T淋巴细胞和噬血细胞巨噬细胞的扩增和活化，与噬血细胞性淋巴组织细胞增生症（HLH）非常相似。这种疾病有很高的发病率和死亡率，并且在识别和开始治疗方面经常存在延迟。在这里，我们将审查什么是已知的发病机制MAS，特别是其相似之处HLH。MAS的发展特征在于细胞因子风暴，伴随着许多促炎细胞因子的产生。我们将研究各种细胞因子在MAS的启动和发病机制中的证据，并讨论新的生物疗法如何改变MAS的风险。最后，我们将审查目前的治疗选择MAS，并研究如何将苦参碱导向治疗作为新的治疗方式。
Macrophage activation syndrome (MAS) is an episode of overwhelming inflammation that occurs most commonly in children with systemic juvenile idiopathic arthritis. It is characterized by expansion and activation of T lymphocytes and hemophagocytic macrophages, and bears great similarity to hemophagocytic lymphohistiocytosis (HLH). This disorder has substantial morbidity and mortality, and there is frequently a delay in recognition and initiation of treatment. Here, we will review what is known about the pathogenesis of MAS and in particular its similarities to HLH. The development of MAS is characterized by a cytokine storm, with the elaboration of numerous proinflammatory cytokines. We will examine the evidence for various cytokines in the initiation and pathogenesis of MAS, and discuss how new biologic therapies may alter the risk of MAS. Finally we will review current treatment options for MAS, and examine how cytokine-directed therapy could serve as novel treatment modalities.