Fatal case of macrophage activation syndrome (MAS) in a patient with dermatomyositis and cytomegalovirus (CMV) viraemia.
Fatal case of macrophage activation syndrome (MAS) in a patient with dermatomyositis and cytomegalovirus (CMV) viraemia.
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DOI：
10.1136/bcr-2018-225231
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发表时间：
2018-07-30
期刊：
影响因子：
0.9
通讯作者：
Barnes, Arti
中科院分区：
文献类型：
--
作者：
Lange, Allison Virginia;Kazi, Salahuddin;Barnes, Arti
我们描述了一例患有皮肌炎（DM）的成人病例，他表现出皮疹、高烧、心动过速和低血压，最初担心是感染性病因或DM发作。她被发现患有巨细胞病毒血症，在开始缬更昔洛韦治疗后有所改善。经过广泛的检查和缺乏改善与广谱抗菌药物治疗，静脉注射免疫球蛋白和类固醇，患者被诊断为巨噬细胞活化综合征后，骨髓活检和可溶性CD 25（可溶性白细胞介素（IL）-2受体）和IL 2的水平。不幸的是，尽管使用地塞米松、阿那白滞素和依托泊苷治疗，患者失代偿，患者家属选择舒适护理。患者随后在重症监护室死亡。
We describe a case of an adult with dermatomyositis (DM) who presents with a rash, high fevers, tachycardia and hypotension, initially concerning for an infectious aetiology or a DM flare. She was found to have cytomegalovirus viraemia which improved after starting valganciclovir. After extensive workup and lack of improvement with broad-spectrum antimicrobial therapy, intravenous immunoglobulin and steroids, the patient was diagnosed with macrophage activation syndrome after bone marrow biopsy and levels of soluble CD25 (soluble interleukin (IL)-2 receptor) and IL2 were obtained. Unfortunately, despite therapy with dexamethasone, anakinra and etoposide, the patient decompensated and the patient's family opted for comfort care. The patient subsequently expired in the intensive care unit.