儿童结缔组织病并间质性肺病53例临床分析

Clinical analysis of 53 children with connective tissue disease complicated with interstitial lung disease

目的探讨结缔组织病(CTD)并间质性肺病(ILD)患儿的临床表现、胸部高分辨率计算机断层扫描(HRCT)变化特点及预后。方法回顾性分析2013年10月至2019年10月西安交通大学附属儿童医院风湿免疫科收治的53例CTD-ILD患儿临床资料,包括其临床表现、血气分析、HRCT、预后等。结果53例CTD-ILD患儿中男女比例为1.0∶1.4;年龄(7.50±3.34)岁;病程2.00(0.85,7.50)个月;其中幼年特发性关节炎(JIA)25例(47.2%),系统性红斑狼疮(SLE)15例(28.3%),多发性肌炎/皮肌炎(PM/DM)11例(20.7%),重叠综合征(OS)1例(1.9%),变应性肉芽肿性血管炎(AGPA)1例(1.9%)。CTD-ILD患儿呼吸系统最常见的症状为咳嗽(39.6%),全身症状最常见为发热(66.0%)。血气分析异常者17例,其中低氧血症10例(18.9%),Ⅰ型呼吸衰竭7例(13.2%)。胸部HRCT表现为磨玻璃影、条索影、胸膜下斑片影、网格影、胸膜增厚、实变影、结节影及囊状低密度影,比例分别为52.8%、26.4%、22.6%、18.9%、11.3%、7.5%、1.9%及1.9%;影像学分型以非特异性间质性肺炎(NSIP)最常见(39.6%)。经糖皮质激素、免疫抑制剂、生物制剂等治疗后,36例(67.9%)胸部HRCT好转,8例(15.1%)无变化,其中75.0%(33/44例)的患儿于治疗过程中发生过感染;9例(17.0%)死亡,原因均为急性呼吸窘迫综合征(ARDS),其中4例为快速进展型,5例为感染继发ARDS。结论CTD-ILD仅少数患儿有呼吸道症状、体征,胸部HRCT有助于CTD-ILD的早期诊断,且影像学表现呈多样性;血气分析、胸部HRCT对于评估病情、选择治疗方案等有指导意义;在积极治疗原发病时,如何有效防治感染,从而提高生存率,减低死亡率,是需要进一步研究的方向。

Objective To investigate the clinical manifestations,characteristics of chest high-resolution computed tomography(HRCT),and prognosis of connective tissue disease(CTD)complicated with interstitial lung disease(ILD)in children.Methods The clinical data of 53 children with CTD-ILD who were admitted to the Department of Rheumatology and Immunology,Affiliated Xi′an Children′s Hospital of Xi′an Jiaotong University from October 2013 to October 2019 were retrospectively analyzed,including clinical manifestations,blood gas analysis,chest HRCT and prognosis.Results As for these 53 children with CTD-ILD,the ratio of male to female was 1.0∶1.4,the average age was(7.50±3.34)years,and the course of disease was 2.00(0.85,7.50)months.Among them,there were 25 cases(47.2%)of juvenile idiopathic arthritis(JIA),15 cases(28.3%)of systemic lupus erythematosus(SLE),11 cases of polymyositis/dermatomyositis(PM/DM)(20.7%),1 case of overlap syndrome(OS)(1.9%),and 1 case of allergic granulomatosis with polyangiitis(AGPA)(1.9%).Although cough(39.6%)was the most common symptom of respiratory system in these children with CTD-ILD and fever(66.0%)was the most common symptom in the systemic features.Blood gas analysis appeared abnormal in 17 cases,including 10 cases of hypoxemia(18.9%)and 7 cases of typeⅠrespiratory failure(13.2%).HRCT chest showed ground glass shadow,strip shadow,subpleural spot shadow,grid shadow,pleural thickening,consolidation shadow,nodular shadow and cystic low-density shadow,with the proportion of 52.8%,26.4%,22.6%,18.9%,11.3%,7.5%,1.9%and 1.9%,respectively;nonspecific interstitial pneumonia(NSIP)(39.6%)was the most common type of imaging classification.After the combined treatment with glucocorticoids,immunosuppressive agents and biological agents,HRCT chest showed remarkably improvement in 36 cases(67.9%),while no change in 8 cases(15.1%).A total of 75.0%(33 cases)of 44 cases were infected in the course of combined treatment.In addition,9 cases(17.0%)died from acute respiratory distress syndrome(ARDS),among which 4 cases exacerbated to rapid progressive luge disease and 5 cases aggravated secondary ARDS due to infection.Conclusions Only a small number of children with CTD-ILD have respiratory symptoms and signs.HRCT chest contributes to the early diagnosis of CTD-ILD,and its imaging manifestations are diverse.Blood gas analysis and HRCT chest play an important role in the disease evaluation and treatment planning.Moreover,it is the direction for further research to develop effective methods to prevent and control secondary infection so as to improve the survival rate and reduce the mortality rate during the active treatment of primary diseases.