摘要目的 对AOSD合并巨噬细胞活化综合征(MAS)的临床资料进行总结,为该病的临床诊断及治疗提供依据.方法 回顾性分析2012年1月至2018年8月在郑州大学第一附属医院住院治疗的AOSD合并MAS患者的临床资料,与同期单纯AOSD的患者进行对比,采用t检验或Mann-Whitney U检验,x2检验或Fisher确切概率法检验进行数据分析.结果 共收集14例AOSD合并MAS患者,占同期AOSD患者的7.6％(14/185),其中男性2例,女性12例,发生MAS时AOSD的中位病程为1.3(0.25,4)个月.与单纯AOSD组相比,合并MAS组发病年龄更小(t=-2.038,P=0.037),出现脾大(t=9.020,P=0.003)、心包积液(t=8.663,P=0.003)、胸腔积液(t=4.754,P=0.029)的比例更高,白细胞计数(t=-4.171,P<0.01)、血红蛋白浓度(t=-2.661,P=0.008)、血小板计数(t=-5.672,P<0.01)、中性粒细胞绝对值(t=-5.082,P<0.01)、白蛋白(t=-3.426,P=0.001)、纤维蛋白原(t=-5.986,P<0.01)、ESR(t=-2.941,P=0.003)、CRP(t=-2.014,P=0.044)显著降低,ALT(t=-3.227,P=0.001)、AST(t=-3.105,P=0.002)、TG(t=-5.612,P<0.01)、铁蛋白>2000μg/L比例(t=7.833,P=0.005)显著增高.14例AOSD合并MAS患者均应用了糖皮质激素,5例应用甲泼尼龙冲击治疗,8例同时应用环孢素A,8例同时应用丙种球蛋白,2例接受依托泊苷,1例接受托珠单抗.治疗后11例好转,3例死亡.结论 年轻的AOSD患者易并发MAS,且在病程早期常见,临床上易出现脾大体征.当AOSD患者出现血细胞计数、纤维蛋白原和ESR进行性降低,TG、铁蛋白进行性升高时常提示MAS的发生,及时治疗可改善患者的预后.

abstractsObjective To summarize the clinical data of macrophage activation syndrome (MAS) in adult-onset Still's disease (AOSD) patients and provide evidence for clinical diagnosis and treatment. Methods We retrospectively reviewed the clinical data of AOSD with MAS patients in the First Affiliated Hospital of Zhengzhou University from January 2012 to August 2018, and compared with patients with AOSD alone. Data were analyzed by t-test, Mann-Whitney U test, x2 test or Fisher exact test. Results A total of 14 AOSD with MAS patients were enrolled, accounting for 7.6％(14/185) of AOSD patients at the same period, including 2 males and 12 females. The median duration of AOSD in MAS was 1.3 (0.25, 4) months. Compared with the AOSD group, the age of onset was younger in the MAS group (t=-2.038, P=0.037), and the proportion of splenomegaly (t=9.020, P=0.003), pericardial effusion (t=8.663, P=0.003), pleural effusion (t=4.754, P=0.029) was higher. The white blood cell count (t=-4.171, P<0.01), hemoglobin level (t=-2.661, P=0.008), platelet count (t=-5.672, P<0.01), neutrophil count (t=-5.082, P<0.01), albumin (t=-3.426, P<0.01), fibrinogen (t=-5.986, P<0.01), ESR (t=-2.941, P=0.003), CRP (t=-2.014, P=0.044) was significantly decreased, ALT (t=-3.227, P<0.01), AST (t=-3.105, P=0.002), triglyceride (t=-5.612, P<0.01), ferritin>2000 μg/L (t=7.833, P=0.005) was significantly increased. Fourteen patients with AOSD complicated with MAS were treated with glucocorticosteroids, 5 with methylprednisolone, 8 with cyclosporine A, 8 with intravenous immunoglobulin (IVIG), 2 with etoposide, and 1 with tocilizumab. After treatment, 11 cases recovered and 3 cases died. Conclusion Younger AOSD patients tend to complicated with MAS, especially at the early course of the disease, and splenomegaly occur more frequently clinically compared to patients without MAS. When blood cell count, fibrinogen and ESR decreases, triglyceride and ferritin levels increases in AOSD patients, the occurrence of MAS is indicated. Timely treatment can improve the prognosis of patients.