Division of Pediatric Rheumatology, Children's Hospital Medical Center, MLC 4010, 3333 Burnet Avenue, Cincinnati, OH 45229, USA.
Best Pract Res Clin Rheumatol. 2014 Apr;28(2):277-92. doi: 10.1016/j.berh.2014.03.002.
Macrophage activation syndrome (MAS) is an episode of overwhelming inflammation that occurs most commonly in children with systemic juvenile idiopathic arthritis (SJIA). 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 pro-inflammatory 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.
巨噬细胞活化综合征 (MAS) 是一种炎症过度活跃的发作，最常见于患有全身型幼年特发性关节炎 (SJIA) 的儿童。它的特征是 T 淋巴细胞和噬血细胞性巨噬细胞的扩增和激活，与噬血细胞性淋巴组织细胞增生症 (HLH) 非常相似。这种疾病具有很高的发病率和死亡率，并且经常存在识别和开始治疗的延迟。在这里，我们将回顾 MAS 的发病机制，特别是其与 HLH 的相似之处。MAS 的发展以细胞因子风暴为特征，产生许多促炎细胞因子。我们将研究各种细胞因子在 MAS 的发生和发病机制中的作用，并讨论新型生物疗法如何改变 MAS 的风险。最后，我们将回顾 MAS 的当前治疗选择，并探讨细胞因子靶向治疗如何作为新的治疗方式。
