目的 分析儿童结缔组织病相关间质性肺病（CTD-ILD）临床资料，提高对儿童CTD-ILD的认识。方法 回顾性分析2012年1月至2021年12月53例CTD-ILD住院患儿临床资料，包括临床表现、胸部HRCT、肺功能、治疗及转归等。结果 53例CTD-ILD患儿中，23例诊断CTD的同时经HRCT评估诊断为ILD，4例以ILD起病，26例CTD诊断ILD的时间跨度为15天~7年余。呼吸系统常见的症状为咳嗽（47.2%）、气促（28.3%）和啰音（26.4%），但有21例患儿无呼吸道症状，依据HRCT诊断ILD。胸部HRCT显示斑片影（64.2%）、条絮/条片影（55.6%）、条索影（37.7%）、网格/蜂窝影（24.5%）、囊状低密度影（24.5%）、磨玻璃影（22.6%）等。肺功能异常以弥散功能下降（46.5%）、限制性通气功能障碍（39.5%）和小气道功能障碍（32.6%）为主。经糖皮质激素、免疫抑制剂、生物制剂治疗后，48例患儿临床症状有好转。胸部HRCT：20例有好转，21例无明显变化，6例进展；肺功能：17例有好转，11例无明显变化，3例进展，8例正常。结论 儿童CTD-ILD临床症状缺乏特异性，胸部HRCT及肺功能检查有助于早期发现肺部病变，需积极完善检查并定期随访；治疗方案主要是糖皮质激素联合免疫抑制剂和/或生物制剂，但生物制剂在儿童中的应用需进一步研究。

Objective To analyze the clinical data of connective tissue disease-associated interstitial lung disease (CTD-ILD) in children, and to improve the understanding of CTD-ILD in children. Methods The clinical data of 53 hospitalized children with CTD-ILD from January 2012 to December 2021 were retrospectively analyzed, including clinical manifestations, chest HRCT, pulmonary function, treatment and outcome, etc. Results Among the 53 children with CTD-ILD, 23 children were diagnosed as ILD by HRCT at the same time of CTD diagnosis, 4 children had ILD onset, and 26 children were diagnosed as ILD with a time span of 15 days to more than 7 years. The common symptoms of respiratory system were cough (47.2%), shortness of breath (28.3%) and rales (26.4%). But there were 21 cases without respiratory symptoms, and ILD was diagnosed according to HRCT. Chest HRCT showed patchy shadows (64.2%), striped shadows (55.6%), streak shadows (37.7%), grid/honeycomb shadows (24.5%), cystic low-density shadows (24.5%) and ground glass shadows (22.6%), etc. The main pulmonary function abnormalities were diffusion dysfunction (46.5%), restrictive ventilation dysfunction (39.5%) and small airway dysfunction (32.6%). After treatment with glucocorticoids, immunosuppressants and biological agents, 48 children showed improvement in clinical symptoms. Chest HRCT showed improvement in 20 children, no significant change in 21 children, and progress in 6 children. Pulmonary function examination showed improvement in 17 children, no significant change in 11 children, progress in 3 children, and normal in 8 children. Conclusions The clinical symptoms of CTD-ILD in children are lack of specificity, and chest HRCT and pulmonary function tests are helpful for early detection of pulmonary lesions, so relevant examinations should be actively completed and regular follow-up should be conducted. Treatment options are glucocorticoids combined with immunosuppressants and/or biological agents, but the use of biological agents in children needs further study.