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

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1、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1LD的临床特点、治疗及预后。结果女性患儿，13岁，GoWrcm’s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，奋所好转。检索国内外报道儿童CADM合并ILD3例

2、，加该患儿共4例，起病年龄10'6岁；首发症状分别为Gottcrn’s征3例、发热2例;4例患儿的抗核抗体及抗Jo_l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1LD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：songhml021@hotmail.com收稿

3、日期：2017-03-09Juvenile-onsetclinicallyamyopathicdermatomyositiscomplicatedwithprogressiveinterstitialpneumonia:reportofonecaseandreviewoftheliteratureWANGHongMAMingshengSONGHongmeiWEIMinDepartmentofPediatrics,PekingUnionMedicalCollegeHospital，PekingUnionMedicalCollege,

4、ChineseAcademyofMedicalSciences;Abstract：ObjectiveToanalyzetheclinicalfeaturesofjuvenile-onsetclinicallyamyopathicdermatomyositiscomplicatedbyprogressiveinterstitialpneumonia.MethodsAretrospectiveanalysisofacaseofjuvenile-onsetclinicallyamyopathicdermatomyositisonclin

5、icalfeatures,diagnosisandtreatmentwasperformed.Dataoftheotherthreereportedcaseswerealsoreviewed.ResultsThepatientwasanadolescentgirlpresentedwithGorrton’ssign.Thepatientdidnothavefatigueandgotnormalresultincreatinekinaseandelctromyogramtest.TheI1RCTexamshowedinterstit

6、ialpneumonia.Themeanageofthefourcasesatthetimeofonsetis12.3yearsold.Gottron’ssign(3/4)andfever(2/4)arethemostcommonsymptomsofonset.Anti-nuclearantibody(ANA)，anti-Jo-1antibodyare100%negativeinthefourpatients.Twoofthefourpatientswhoreceivedanti-Ro-52antibodytestarcbothp

7、ositive.ThreeofthefourpatientswereasymptomaticwhentheCTscanshowedinterstitialpneumonia.Theinterstitialpneumoniawasprogressiveandthreeofthefourpatientsdiedofrespiratoryfailurewithinsixmonths.Treatmentwithglucocorticoidandimmunosuppressantwassuccessfulinonecase.Conclusi

8、onsJuvenileCADMcanbecomplicatedbyprogressiveinterstitialpneumonia.ChildrensuspectedCADMshouldperformpulmonaryimagingexaminat