肺间质疾病(ILD)是一组病因广泛的异质性疾病,婴幼儿时期特殊类型的ILD(DILD)病因分类更为复杂。结缔组织病(CTD)是一组累及全身多系统、多脏器的自身免疫性疾病,肺部为最常累及的脏器之一,结缔组织病相关肺间质疾病(CTD-ILD)与ILD有相似的肺部临床症状,亦有各自特点,但治疗方案及预后截然不同,加强对婴幼儿CTD-ILD与ILD的认识,学习其临床分析思路,对开展广泛深入的临床研究很有必要。 Pulmonary interstitial disease (ILD) is a group of heterogeneous diseases with a wide range of causes. The specific type of ILD (DILD) in infancy is more complicated. Connective tissue disease (CTD) is a group of autoimmune diseases involving the whole body, multiple systems and multiple organs. The lungs are one of the most frequently involved organs. CTD-ILD associated with ILD There are similar clinical symptoms of the lungs, but also have their own characteristics, but the treatment plan and prognosis are very different. It is necessary to strengthen understanding of CTD-ILD and ILD in infants and young children and to learn the clinical analysis of them.