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| 成人Still病合并巨噬细胞活化综合征13例临床特征分析 | |
| 引用本文： | 武加标,施冶青,赵东宝. 成人Still病合并巨噬细胞活化综合征13例临床特征分析[J]. 中华全科医学, 2020, 18(3): 423-426. DOI: 10.16766/j.cnki.issn.1674-4152.001263 |
| 作者姓名： | 武加标 施冶青 赵东宝 |
| 作者单位： | 1. 徐州医科大学附属武进临床学院 常州市武进人民医院风湿科, 江苏 常州 213002; |
| 基金项目： | 上海领军人才队伍建设专项基金(201444) |
| 摘 要： | 目的 对成人Still病(adult-onset Still's disease,AOSD)并发巨噬细胞活化综合征(macrophage activation syndrome,MAS)的临床特点、实验室检查及治疗和转归进行分析,旨在尽早识别该病,提高诊治水平。 方法 回顾性分析2011年7月—2015年8月上海长海医院风湿免疫科住院诊断为AOSD并MAS的13例患者的临床资料。 结果 13例患者,其中男性3例,女性10例,平均年龄(44.1±16.9)岁,病程0.4~60个月不等。13例AOSD患者发生MAS时,临床主要特征:高热12例(92%)、铁蛋白增高(>2 000μg/L)12例(92%),发生皮疹11例(85%),肝功能损伤10例(77%),关节疼痛7例(54%),多发淋巴结肿大7例(54%),咽痛6例(46%),肝脾肿大4例(31%),血液系统受累,贫血11例(84%),两系减少8例(62%),呼吸系统以胸腔积液居多(31%)。AOSD并发MAS时,骨髓细胞学明确嗜血现象有4例,治疗上13例患者均加用糖皮质激素,11例加用环孢素A,6例加用丙种球蛋白,8例患者经治疗后缓解出院,5例死亡,死因:肺部混合感染出现急性心肺循环衰竭1例,间质性肺炎伴感染导致呼衰1例,急性肺水肿并心衰、肺部感染1例,多脏器功能衰竭2例。 结论 AOSD出现持续高热、红细胞沉降率(erythrocyte sedimentation rate,ESR)下降和纤维蛋白原减低可能提示并发MAS,感染可能是AOSD并发MAS的诱因之一,糖皮质激素联合环孢素(CsA)治疗可抢先控制疾病的发生发展。 |
| 关 键 词： | 成人Still病 巨噬细胞活化综合征 反应性嗜血细胞综合征 幼年类风湿关节炎全身型 |
| 收稿时间： | 2018-09-11 |
Clinical analysis of 13 cases macrophage activation syndrome complicating adult-onset Still's disease |
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| Affiliation: | 1. Department of Rheumatology, Changzhou Wujin People's Hospital, Changzhou, Jiangsu 213002, China |
| Abstract: | Objective To analyze the clinical characteristics and laboratory data of Adult-onset Still's Disease(AOSD) complicated with macrophage activation syndrome(MAS). Methods A retrospective analysis of 13 cases of AOSD, complicated by MAS diagnosed in Changhai Hospital of Shanghai from July 2011 to August 2015. Results Among 13 patients, 3 cases were male, 10 cases were female, age(44.1±16.9) years. The main clinical features were fever, high ferritin(more than 2 000 μg/L) with 92% incidence rate(n=12).Rash occurred rate was 85%(n=11) and liver function damage incidence(n=10) 77%, joint pain 54%(n=7), multiple lymph node enlargement of 54%(n=7), incidence of sore throat was 46%(n=6), hepatosplenomegaly incidence rate of 31%(n=4), blood system involvement, anemia incidence rate was 85%(n=11). Blood cells decreased 62%(n=8), when MAS occurs, bone marrow cytology clearly is seen in 3 cases, the treatment of glucocorticoid and CsA delays the disease process. All of the 13 patients were treated with glucocorticoid, 11 cases were treated with cyclosporine, 6 cases were treated with immunoglobulin, 62%(8/13) patients after treatment was alleviated, 5 cases died. The causes of death include mixed infection of the lungs appeared acute cardiopulmonary circulation failure(1 case), interstitial pneumonia associated with infection leaded to respiratory failure(1 case), acute pulmonary edema, heart failure and pulmonary infection after hospital discharge(1 case), multiple organ dysfunction syndrome(1 case), pneumonia associated with infection leaded to respiratory failure(1 case). Conclusion It indicates that MAS complicated when AOSD has high fever, ESR and fibrinogen decreased. Guarding against that infection is the cause of AOSD to induce MAS. Glucocorticoid combined with IVIG and CsA can control the disease development. |
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