Abstract:
Patients with established rheumatic disorders may develop complications of macrophage activation syndrome due to severe flares of the underlying disease (adult-onset Still\'s disease, SLE); however, in most other rheumatic disorders, MAS develops in association with identified viral or other infectious triggers. It is therefore important to pursue appropriate studies to identify potential infectious triggers in rheumatic disease patients who develop MAS. Management is best directed toward treatment of the triggering infections and combinations of high-dose corticosteroids, calcineurin inhibitors, and biologic therapies targeting IL-1 and/or IL-6 to suppress the associated cytokine storm.
摘要:
由于潜在疾病的严重耀斑(成人发作的斯蒂尔病,SLE);然而,在大多数其他风湿病中,MAS与已识别的病毒或其他感染性触发因素相关地发展。因此,重要的是进行适当的研究,以确定发生MAS的风湿性疾病患者的潜在感染诱因。管理最好针对触发感染的治疗和高剂量皮质类固醇的组合,钙调磷酸酶抑制剂,以及靶向IL-1和/或IL-6的生物疗法,以抑制相关的细胞因子风暴。