儿童皮肌炎回顾性研究——53例临床分析  

A retrospective study of juvenile dermatomyositis: clinical analysis of 53 cases

作　　者：余红[1,2] 李春晓[2] 姜承易 李玉华[3] 李化国 程茹虹 张珍[2] 赵东宝[4] 

机构地区：[1]第二军医大学研究生院临床医学系,上海200433 [2]上海交通大学医学院附属新华医院皮肤科,上海200092 [3]上海交通大学医学院附属新华医院放射科,上海200092 [4]第二军医大学附属长海医院风湿免疫科,上海200433

出　　处：《诊断学理论与实践》2014年第1期49-53,共5页Journal of Diagnostics Concepts & Practice

摘　　要：目的:分析53例儿童皮肌炎(juvenile dermatomyositis,JDM)患者的病史资料,以加强临床对该病的认识。方法:对2001年至2012年的53例病史资料完整的JDM患者进行回顾性分析,内容包括临床表现、实验室检查、治疗方案及近期随访情况。结果:53例患儿中面部水肿性红斑发生率达98%,Gottron丘疹为89%,甲周毛细血管扩张性红斑为30%,钙盐沉积为6%。肌力检测下降者有66%;肌酸激酶同工酶MB(creatine kinase MB,CK-MB)异常升高达70%;肌电图检测率为57%,其中73%者结果异常;肌肉活检检测率为10%,结果均为异常;MRI检测率为42%,检出异常者占78%。所有患者都接受了激素治疗,其中40%加用了丙种球蛋白静脉滴注后好转。24例平均随访7.7年,其中75%的病例症状部分缓解。结论:MRI检查能灵敏而无创地显示出JDM的肌肉病变。丙种球蛋白辅助治疗可减少患者激素的用量,且应用相对安全。JDM病程大多为慢性,需要长期观察。Objective： To study the clinical features of juvenile dermatomyositis （JDM） for better understanding of the disease. Methods： Fifty-three in-hospital patients with JDM during 2001-2012 were enrolled. Clinical symptoms, lab- oratory data, treatment and outcome were reviewed. Results： Clinical manifestations of these patients included malar rash （98%）, Gottron＇s papules （89%）, periungual erythema（30%）, calcinosis （6%）, and muscles weakness（66%）. Elevated serum level of CK-MB was found in 70% of the patients. Electromyography was performed in 57% of the patients and 73% of them showed abnormal results; 10% of the patients accepted muscle biopsy and 100% of them were abnormal. Magnetic Resonance Imaging （MRI） was applied in 42% of the patients, and 78% of them presented muscle abnormality. All the patients were treated with corticosteroids, and 40% of the patients were given additionally gamma globulin intravenously. Twenty-four patients got a average follow-up of 7.7 years, and 75% of them got partial remission. Conclusion： As a non- invasive imagine technique, MRI could sensitively reveal the muscle changes in JDM. Intravenous gamma globulin com- bined with eorticosteroids could be effective and relatively safe therapy for the intractable disease, and reduce the dosage of the steroids. Mostly, JDM is a chronic process requiring a long-term observation.

关 键 词：儿童皮肌炎 临床特点 磁共振 

分 类 号：R593.2[医药卫生—内科学]