重症成人Still病1例报道并文献复习

doi:10.3969/j.issn.1002-1949.2018.08.012

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摘要目的探讨成人Still病（adult onset Still′s disease，AOSD）继发噬血细胞综合征（hemophagocytic syndrome，HPS）的临床资料及预后。方法以“adult onset Still′s disease”、“macrophage activation syndrome”、“hemophagocytic lymphohistiocytosis” 和 “hemophagocytic syndrome”为检索词检索PubMed数据库，以“（成人斯蒂尔病 or 成人 Still′s病）、（巨噬细胞活化综合征 or 嗜血细胞综合征 or 噬血细胞综合征）”为检索词检索CNKI、WangFang Data和VIP数据库，时限为建库~2016年。结果患者，女，34岁，发热伴皮疹、咽痛、关节痛半个月,于2017-10-04入院。初步诊断发热待查，予以多种抗菌药物治疗后体温持续8 d未见下降，甲强龙使用48 h后体温再次上升，且出现血压下降、呼吸困难，骨穿见嗜血细胞，诊断AOSD、HPS、多脏器功能障碍综合征（MODS）。经地塞米松、环孢素（cyclosporine A, CsA）、依托泊苷（etoposide, VP16）治疗好转。检索文献54篇，其中5例因数据不全被排除，余下的49例，女39例，男10例，平均年龄（34.8±13.4）岁。HPS临床表现中发热、脾大、吞噬现象发生率高，血小板减少、铁蛋白、肝酶增高显著。HPS患者激素用量多，CsA、丙种球蛋白（IVIG）使用比例高，4例接受VP16治疗。11例死亡，5例死于MODS，3例死于中枢神经系统感染，余3例分别死于肠出血、呼吸衰竭、间质性心肌炎。结论 HPS诊治关键在于早期诊断；早期激素联合免疫抑制剂治疗AOSD继发HPS（如出现持续血三系下降，积极应用VP16），合并MODS预后差。

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	王敏
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关键词 ：成人Still病(AOSD）, 嗜血细胞活化综合征（HPS）, 多脏器功能障碍综合征（MODS）, 依托泊苷（VP16）

基金资助:国家自然科学青年研究基金（81601661）；安徽省自然科学基金面上项目（1608085MH195）

通讯作者: 杨旻（1978-），女，博士，副教授，主任医师，E-mail:456ym@163.com。

作者简介: 王敏（1992-），女，硕士研究生，住院医师，E-mail:1508093142@qq.com。

引用本文:

王敏，郑瑶，杨旻. 重症成人Still病1例报道并文献复习[J]. 中国急救医学, 2018, 38(8): 700-705.
Wang Min, Zheng Yao, Yang Min. Severe adult onset Still′s disease: a case report and literature review. Chinese Journal of Critical Care Medicine, 2018, 38(8): 700-705.

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http://www.cnemergency.org.cn/CN/10.3969/j.issn.1002-1949.2018.08.012 或 http://www.cnemergency.org.cn/CN/Y2018/V38/I8/700

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