CAJ | 학술논문

目的探讨合并间质性肺疾病(ILD)的儿童皮肌炎(JDM)患儿的临床、实验室特点以及转归.方法回顾性分析2005年1月至2011年12月,在北京儿童医院住院的110例JDM中合并ILD的39例患儿临床资料,对其确诊时的临床表现、实验室检查及其转归进行回顾性分析.结果 39例中男16例,女23例,男:女=1∶1.4；平均发病年龄为5.6岁.发病年龄小于6岁的患儿24例(61.5％).首发症状依次为单纯皮疹(17例,43.6％)、皮疹和肌肉症状同时出现(14例,35.9％)、发热(4例,10.1％)以及肌无力(3例,7.7％).仅有20例(51.3％)的患儿有呼吸系统症状,但同时合并有消化系统症状的患儿24例(61.5％),合并心电图及心脏彩超异常的患儿27例(69.2％).患儿胸部CT肺部有多种改变,均有肺间质改变,有条索状改变25例次(64.1％),为磨玻璃改变10例次(25.6％),网、线状影9例次(23.1％)及结节改变5例次(12.8％).同时合并有肺实质浸润的比例高(28例,71.8％).病变部位以双肺背侧为主.严重者(4例,10.1％)可合并纵隔气肿、气胸、肺出血及皮下气肿.24例患儿行肺功能检查,异常者15例(62.5％).本组资料病死4例(10.3％).结论 JDM合并ILD患儿的肺部CT检查往往早于临床症状发现ILD,多与其他系统、脏器损害并存.预后较未合并ILD的JDM患儿差.对于起病年龄早,合并多脏器损害的患儿应早期完善肺CT等相关检查并早期及时治疗.
목적 탐토합병간질성폐질병(ILD)적인동피기염(JDM)환인적림상、실험실특점이급전귀.방법 회고성분석2005년1월지2011년12월,재북경인동의원주원적110례JDM중합병ILD적39례환인림상자료,대기학진시적림상표현、실험실검사급기전귀진행회고성분석.결과 39례중남16례,녀23례,남:녀=1∶1.4；평균발병년령위5.6세.발병년령소우6세적환인24례(61.5％).수발증상의차위단순피진(17례,43.6％)、피진화기육증상동시출현(14례,35.9％)、발열(4례,10.1％)이급기무력(3례,7.7％).부유20례(51.3％)적환인유호흡계통증상,단동시합병유소화계통증상적환인24례(61.5％),합병심전도급심장채초이상적환인27례(69.2％).환인흉부CT폐부유다충개변,균유폐간질개변,유조색상개변25례차(64.1％),위마파리개변10례차(25.6％),망、선상영9례차(23.1％)급결절개변5례차(12.8％).동시합병유폐실질침윤적비례고(28례,71.8％).병변부위이쌍폐배측위주.엄중자(4례,10.1％)가합병종격기종、기흉、폐출혈급피하기종.24례환인행폐공능검사,이상자15례(62.5％).본조자료병사4례(10.3％).결론 JDM합병ILD환인적폐부CT검사왕왕조우림상증상발현ILD,다여기타계통、장기손해병존.예후교미합병ILD적JDM환인차.대우기병년령조,합병다장기손해적환인응조기완선폐CT등상관검사병조기급시치료.
Objective To explore the clinical and laboratory features and the prognosis of juvenile dermatomyositis (JDM) complicated with interstitial lung disease(ILD).Method Data of 39 cases of JDM complicated with ILD hospitalized in Beijing Children's Hospital from January 2005 to December 2011 were collected.The clinical features,laboratory data and prognosis of these children were analyzed.Result Of the 39 cases studied,16 were boys,and 23 girls.The average age of onset was 5.6 years,and 61.5％ of the patients' age of onset(24 cases) was under 6 years.Rashes( 17 cases,43.6％ ),simultaneous eruption of rashes and muscle weakness( 14 cases,35.9％ ),fever(4 cases,10.1％ ),or muscle weakness(3 cases,7.7％ ) were common initial symptoms of the disease. Only 51.3％ of the patients (20 cases)had the symptoms of respiratory system,but (24 cases)61.5％ were complicated with that of the gastrointestinal system;( 27 cases)69.2％ had at the same time electrocardiographic and echocardiographic abnormalities.The chest high resolution computed tomography (HRCT) showed cord or baud-like shadows in their lungs of more than half of the cases (25 cases,64.1％ ),and other changes included ground glass-like shadow ( 10 cases,25.6％ ),net and lineation-like shadow(9 cases,23.1％ ),nodular change(5 cases,12.8％ ).The patients complicated with lung essential infiltration accounted for as high as 71.8％ (28 cases).These imaging changes were largely seen on both dorsal sides of their lungs.Severe patients also had mediastinal emphysema,pneumothorax,pneumorrhagia or aerodermectasia Twenty-four patients underwent pulmonary function examination,and 62.5％ of the patients' pulmonary function ( 15 cases)was abnormal.The fatality rate of the cases studied was 10.1％.Conclusion The imaging changes of patients suffering from JDM with ILD were often more severe as compared to the clinical symptoms,and were often complicated with damages to other systems and organs.The prognosis of those patients was poorer than others.Patients with JDM especially at a younger age of onset and with various organ damages should be examined with chest HRCT examinations as early as possible.