幼年皮肌炎复发的临床特点及相关危险因素分析  

Clinical characteristics and risk factors of juvenile dermatomyositis with relapses

作　　者：樊志丹[1] 马慧慧[1] 郭翼红[1] 张雅媛[1] 俞海国[1] Fan Zhidan;Ma Huihui;Guo Yihong;Zhang Yayuan;Yu Haiguo(Department of Rheumatology and Immunology,Children's Hospital of Nanjing Medical University,Nanjing 210008,China)

机构地区：[1]南京医科大学附属儿童医院风湿免疫科,南京210008

出　　处：《中华风湿病学杂志》2022年第6期367-372,共6页Chinese Journal of Rheumatology

基　　金：国家自然科学基金(81771762,81202345);江苏省六大人才高峰项目(WSW-089)。

摘　　要：目的分析幼年皮肌炎(JDM)患儿复发的临床资料、实验室检查特征及预测复发的危险因素。方法回顾性分析2017年3月至2021年3月在南京医科大学附属儿童医院住院诊治的JDM患儿临床资料,患儿分为单病程组和复发组,比较2组患儿病程、临床表现、实验室检查、治疗转归等情况。计量资料组间比较应用t检验或者Mann-WhitneyU检验,计数资料组间比较应用χ^(2)检验或Fisher确切概率法,并用多因素Logistic回归分析影响JDM患儿复发的危险因素。结果102例JDM患儿中20例(19.6%)在药物减量过程中或停药后出现疾病复发,复发中位时间为3.24年(9个月至7年)。复发组患儿中肌炎特异性抗体(MSA)阳性率为40%(8/20),其中5例患儿抗核基质蛋白2抗体阳性,2例抗转录中介因子1-γ抗体阳性,1例抗信号识别颗粒抗体阳性,其余12例均为MSA阴性。2组患儿的临床资料和实验室检查项目中,皮下钙化[OR值(95%CI)=17.54(1.55,198.64),P=0.021]及肺间质病变[OR值(95%CI)=3.83(1.27,11.59),P=0.017]是JDM患儿复发的独立危险因素。102例患儿中53例(51.9%)初始治疗给予甲泼尼龙冲击,复发组患儿13例(65.0%)初始治疗给予甲泼尼龙冲击,2组比较差异无统计学意义(χ^(2)=1.70,P=0.193)。肿瘤坏死因子拮抗剂联合甲氨蝶呤可作为复发患儿的选择,在临床治疗中取得良好的效果。结论JDM患儿复发率高,皮下钙化和肺间质病变是患儿复发的危险因素,复发组患儿经积极治疗仍可获得满意的治疗效果。Objective To analyze the clinical characteristics and risk factors of juvenile dermatomyositis(JDM)with relapses by comparing clinical features,treatment and disease course among JDM patients with and without relapses.Methods A retrospective analysis of 102 JDM patients from Children's Hospital of Nanjing Medical University between March 2017 and March 2021 was carried out.Patients were divided into two groups based on whether a JDM relapse had occurred or not.Initial clinical features,laboratory tests and treatment were compared between the two groups.T-test or Mann-Whitney U test was used for measurement data,chi-square test or fisher exact probability was used for count data.The features associated with risk of relapses were analyzed by multivariate logistic regression.Results Among 102 children with JDM,twenty patients(19.6%)relapsed during drug reduction or after drug withdrawal.The mean duration to the first relapse was 3.24 years(range:9 months to 7 years).Myositis specific antibodies(MSA)were positive for 8(40.0%)patients with relapses.With 5 cases were anti-nuclear matrix protein 2 positive,2 cases were anti-transcription interme-diary factor 1 gamma positive,1 case was anti-signal recognition particle(SRP)positive,the other 12 cases were MSA negative.By binary logistic regression analysis,we found that peripheral calcinosis[OR(95%CI)=17.54(1.55,198.64),P=0.021],and interstitial lung disease[OR(95%CI)=3.83(1.27,11.59),P=0.017]were independently related to JDM with relapses.Fifty-three patients(51.9%)received methylpre-dnisolone pulse therapy for initial treatment and 13(65.0%)patients with relapses received methylprednisolone pulse for initial treatment.There was no significant difference between the two groups(χ^(2)=1.70,P=0.193).Tumor necrosis factor alpha antagonist combined with methotrexate(MTX)had achieved good results in clinical treatment in children with relapses.Conclusion The risk of relapses is high in children with JDM.Calcinosis and interstitial lung disease at disease onset can predict a

关 键 词：皮肌炎 复发 危险因素 皮下钙化 肺间质病变 

分 类 号：R725.9[医药卫生—儿科]