上海交通大学学报（医学版）

上海交通大学学报（医学版） ›› 2017, Vol. 37 ›› Issue (6): 780-.doi: 10.3969/j.issn.1674-8115.2017.06.012

• 论著（临床研究） • 上一篇    下一篇

成人斯蒂尔病继发巨噬细胞活化综合征的临床分析

涂洋 *，王然 *，郭茹茹，王苏丽，扶琼，吕良敬   

  1. 上海交通大学 医学院附属仁济医院风湿科，上海 200001
  • 出版日期:2017-06-28 发布日期:2017-07-05
  • 通讯作者: 吕良敬，电子信箱：lu_liangjing@medmail.com.cn
  • 作者简介:涂洋（1988—），男，博士生；电子信箱：tuyangzzu@126.com。王然（1992—），女，博士生；电子信箱：wangran686@126.com。* 共同第一作者。
  • 基金资助:

    上海市教育委员会高峰高原学科建设计划（20152214）；国家重点研发计划项目（SQ2017YFSF090114）；国家自然科学基金（81373209）

Clinical characteristics of patients with macrophage activation syndrome secondary to adult-onset Still’s disease

TU Yang*, WANG Ran*, GUO Ru-ru, WANG Su-li, FU Qiong, LÜ Liang-jing   

  1. Department of Rheumatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200001, China
  • Online:2017-06-28 Published:2017-07-05
  • Supported by:

     Shanghai Municipal Education Commission—Gaofeng Clinical Medicine Grant Support, 20152214; National Key R&D Plan, SQ2017YFSF090114; National Natural Science Foundation of China, 81373209

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摘要：

目的 · 对成人斯蒂尔病（AOSD）合并巨噬细胞活化综合征（MAS）患者的临床资料进行分析总结，为该病的临床诊断和 治疗提供依据。方法 · 收集上海交通大学医学院附属仁济医院2013 年 1 月至2016 年 6 月收治的42 例 AOSD 患者，其中14 例为 AOSD 合并 MAS 患者（MAS 组）， 28 例为性别、年龄匹配但未发生 MAS 的 AOSD 患者（非 MAS 组）；对临床资料进行回顾性分析。 结果 · MAS 组与非 MAS 组患者在性别构成、年龄、病程方面的差异均无统计学意义。与非 MAS 组比较，MAS 组患者的病死率及皮 疹、脾肿大、吞噬现象的发生率较高；血清谷丙转氨酶（ALT）、铁蛋白显著增高，纤维蛋白原（FDP）显著降低；在治疗方面，MAS 组患者激素用量较大，甲氨蝶呤使用比例较低，丙种球蛋白使用比例较高，有 2 例接受依托泊苷治疗。结论 · 与非 MAS 组患者比较， MAS 组患者的病死率较高；临床表现中皮疹、脾脏肿大、吞噬现象的发生率较高；血清铁蛋白、ALT 显著升高，FDP 显著降低。早 期诊断积极治疗是改善预后的关键。

关键词: 成人斯蒂尔病, 巨噬细胞活化综合征, 铁蛋白

Abstract:

Objective · To investigate the clinical features of macrophage activation syndrome (MAS) associated with adult-onset Still's disease (AOSD), and provide the basis for clinical diagnosis and treatment of the disease.  Methods · The clinical data of 42 patients with AOSD, including 14 patients with AOSD-induced MAS (the MAS group) and 28 AOSD patients paired by age and sex (the non-MAS group), diagnosed in Department of Rheumatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine from October 2013 to June 2016 were collected and then retrospectively analyzed.  Results · There was no significant difference in age, sex and duration of AOSD between two groups. The mortality rate of patients in MAS group was significantly higher than that of patients in non-MAS group, as well as the rates of rash, splenomegaly and hemophagocytosis. The levels of ALT and serum ferritin in MAS group were higher than those in non-MAS group, while the level of FDP is lower. Glucocorticoids were used in all 42 patients, and the dosage of glucocorticoids was significantly higher in MAS group than non-MAS group. Only 1 patient with AOSD-induced MAS received MTX, the percentage of patients receiving MTX was significantly lower in MAS group than non-MAS group. Five patients with AOSD-induced MAS received IVIG, the percentage of patients receiving IVIG was significantly higher in MAS group than non-MAS group. Two patients with AOSD-induced MAS received VP-16.  Conclusion · The mortality rate of patients in MAS group was significantly higher than that of patients in non-MAS group, as well as the rates of rash, splenomegaly and hemophagocytosis. The levels of ALT and serum ferritin in patients with AOSD-induced MAS were higher than patients without MAS, while the level of FDP was lower. Early diagnosis and active treatment is the key point to improve clinical outcome.

Key words: adult-onset Still's disease, macrophage activation syndrome, ferritin