儿童临床无肌病皮肌炎合并肺间质病变1例报告及文献复习.docVIP

儿童临床无肌病皮肌炎合并肺间质病变1例报告及文献复习 王红 马明圣 宋红梅 魏珉 中国医学科学院北京协和医学院北京协和医院儿科 X 关注成功！ 加关注后您将方便地在 我的关注中得到本文献的被引频次变化的通知！ 新浪微博 腾讯微博 人人网 开心网 豆瓣网 网易微博 摘????要： 目的 分析儿童起病的临床无肌病皮肌炎 (CADM) 合并肺间质病变 (ILD) 的临床特点。方法 回顾1例成功治疗的CADM合并ILD患儿的临床资料, 并检索中英文数据库, 分析儿童CADM合并ILD的临床特点、治疗及预后。结果 女性患儿, 13岁, Gottrons征阳性, 临床无乏力症状, 肌酸激酶、肌电图均正常, 胸部高分辨CT提示肺间质性病变。经积极加用糖皮质激素联合环磷酰胺治疗, 有所好转。检索国内外报道儿童CADM合并ILD 3例, 加该患儿共4例, 起病年龄1016岁;首发症状分别为Gotterns征3例、发热2例;4例患儿的抗核抗体及抗Jo-1抗体均为阴性, 2例抗Ro-52抗体阳性。3例文献报道的患儿在发现ILD时无呼吸系统临床症状, 半年内迅速进展为呼吸衰竭死亡。结论 儿童CADM合并ILD可呈现为快速进展型肺间质性病变, 临床疑诊为CADM的患儿应完善肺部影像学检查, 如存在ILD应予糖皮质激素联合免疫抑制剂积极治疗, c改善预后。 关键词： 临床无肌病皮肌炎; 肺间质病变; 肌酸激酶; 儿童; 作者简介：宋红梅, 电子信箱:songhm1021@ 收稿日期：2017-03-09 Juvenile-onset clinically amyopathic dermatomyositis complicated with progressive interstitial pneumonia: report of one case and review of the literature WANG Hong MA Mingsheng SONG Hongmei WEI Min Department of Pediatrics, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences; Abstract： Objective To analyze the clinical features of juvenile-onset clinically amyopathic dermatomyositis complicated by progressive interstitial pneumonia. Methods A retrospective analysis of a case of juvenile-onset clinically amyopathic dermatomyositis on clinical features, diagnosis and treatment was performed. Data of the other three reported cases were also reviewed. Results The patient was an adolescent girl presented with Gorrtons sign. The patient did not have fatigue and got normal result in creatine kinase and elctromyogram test. The HRCT exam showed interstitial pneumonia. The mean age of the four cases at the time of onset is 12. 3 years old. Gottrons sign ( 3/4) and fever ( 2/4) are the most common symptoms of onset. Anti-nuclear antibody (ANA) , anti-Jo-1 antibody are 100 % negative in the four patients. Two of the four patients who received anti-Ro-52 antibody test are both positive. Three of the four patients were asymptomatic when the CT scan showed interstitial pneumon