Worsening of macrophage activation syndrome in a patient with adult onset Still's disease after initiation of etanercept therapy
Worsening of macrophage activation syndrome in a patient with adult onset Still's disease after initiation of etanercept therapy
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DOI：
10.1097/00124743-200108000-00013
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发表时间：
2001-08-01
影响因子：
3.4
通讯作者：
Buckley, L
中科院分区：
文献类型：
--
作者：
Stern, A;Riley, R;Buckley, L
巨噬细胞活化综合征（MAS）是一种罕见的，潜在的致命的，临床综合征，这已被描述在儿童风湿性疾病。MAS定义为长期发热、全血细胞减少和高纤维蛋白血症（伴或不伴低纤维蛋白原血症）。骨髓、脾或淋巴结的组织学检查显示单核吞噬细胞的噬血作用。在斯蒂尔病患者中，观察到的NIAS触发事件包括感染过程和药物，如胃肠外金和非甾体抗炎药。我们报告的情况下，一个年轻女子与成人发病斯蒂尔斯病（AOSD），并发EB病毒感染和随后的MAS，其过程中恶化后，管理的可溶性肿瘤坏死因子-1 α受体，依那西普。随后的冲击皮质类固醇和环孢素A治疗引起了显着的临床改善。缺乏关于使用依那西普治疗AOSD的治疗数据;鉴于我们的经验和最近在Still病儿童中的报告，我们建议在AOSD患者中谨慎使用该药物，特别是当并发MAS时。
The macrophage activation syndrome (MAS) is a rare, potentially fatal, clinical syndrome, which has been described in childhood rheumatic disorders. MAS is defined by a prolonged period of fevers, pancytopenia, and hypertriglyceridemia (with or without hypofibrinogenemia). Histopathological examination of bone marrow, spleen, or lymph nodes shows hemophagocytosis by mononuclear phagocytes. In patients with Still's disease, observed triggering events for NIAS have included both infectious processes and pharmacological agents, such as parenteral gold and nonsteroidal anti-inflammatory drugs. We report the case of a young woman with adult-onset Stills disease (AOSD), complicated by an Epstein-Barr virus infection and subsequent MAS, whose course worsened after administration of the soluble tumor necrosis factor-1 alpha receptor, etanercept. Subsequent treatment with pulse corticosteroids and cyclosporine A induced a dramatic clinical improvement. Treatment data regarding the use of etanercept in AOSD axe lacking; given our experience and recent reports in children with Still's disease, we would suggest caution using this agent in patients with AOSD, particularly when complicated by MAS.