幼年皮肌炎的临床特点  

CLINICAL FEATURES OF JUVENILE DERMATOMYOSITIS

作　　者：付元[1] 常红[1] 陈秀霞[1] 吴蒙蒙[1] 

机构地区：[1]青岛大学医学院附属医院儿内科,山东青岛266003

出　　处：《青岛大学医学院学报》2014年第1期64-67,共4页Acta Academiae Medicinae Qingdao Universitatis

摘　　要：目的 探讨幼年皮肌炎（J D M）的临床特点、治疗和预后,以提高诊疗水平。方法 回顾性分析19例J D M病儿的临床资料。结果 19例JD M病儿中,男8例,女11例,发病年龄1岁10月～15岁,中位数年龄10岁;19例病儿有皮疹和肌肉症状,其中并发肺炎2例,免疫相关性甲状腺炎1例,嗜血细胞综合征1例。实验室检查14例病儿肌酸激酶（CK）升高;肌电图示肌源性损害13例;3例肌肉活检示肌炎改变。所有病儿均使用糖皮质激素治疗,6例病儿加用甲氨蝶呤;除1例病儿因嗜血细胞综合征死亡,余病情好转出院。12例病儿出现误诊,误诊为过敏性皮炎、关节炎、呼吸道感染等。结论 JDM是一种发病率较低的疾病,早期发病较隐匿,易误诊,可累及多个器官,其中以呼吸系统受累多见。Objective To investigate the clinical features, treatment and prognosis of juvenile dermatomyositis （JDM） so as to improve the quality in the management of this condition. Methods A retrospective analysis was done 19 patients with JDM. Results Of the 19 sick kids, eight were boys, and 11 girls, age of onset：22 months to 15 year-old, median age being 10 years old. All the patients had skin rash and myositis, of whom, two complicated with pneumonia, one with immune related thyroiditis, and one with hemophagocytic syndrome. Laboratory examination： ereatinephosphate kinase （CK） elevated in 14 patients, and 13 elec- tromyograms revealed myogenic damage; three muscle biopsies showed myositis. Corticosteroid was used for all the patients, and rnethotrexate was added in six of them. One died of hemophagoeytic syndrome, the others improved and discharged. Diagnostic errors were seen in 12 patients, who were misdiagnosed as having atopic dermatitis,arthritis or respiratory tract infections. Conclusion JDM is a disease of lower incidence, the onset is insidious in the early stage, and misdiagnosis is commonly seen. Multiple organs can be involved, respiratory system in particular.

关 键 词：皮肌炎 治疗学 诊断 鉴别 

分 类 号：R593.26[医药卫生—内科学]