摘要肺纤维化(PF)是一种病因不明的慢性间质性肺病，在儿童中较少见，但作为免疫出生缺陷(IEI)较常见的肺部并发症，已逐渐引起人们的关注。PF常见于IEI中的伴典型表现的联合免疫缺陷综合征、抗体免疫缺陷病、免疫失调性疾病、吞噬细胞缺陷、自身炎症性疾病及骨髓衰竭性疾病。IEI患者合并PF通常发病年龄较小，临床表现缺乏特异性，胸部CT表现常不典型，且传统治疗方案效果差。现就IEI合并PF的致病机制、临床特征及治疗方法进行综述，旨在提高儿科医师对儿童IEI合并PF的早期识别与靶向治疗水平。

abstractsPulmonary fibrosis (PF) is a chronic interstitial lung disease of unknown etiology, which is rare in children but has garnered increasing attention as a common pulmonary complication in inborn errors of immunity (IEI). PF is most frequently observed in IEI subtypes such as combined immune deficiencies with syndromic features, antibody deficiencies, immune dysregulation disorders, phagocytic defects, autoinflammatory diseases, and bone marrow failure syndromes.Pediatric patients with IEI-associated PF typically present at a younger age, with nonspecific clinical manifestations, atypical findings on chest computed tomography, and limited response to conventional therapies.In this review, the pathogenic mechanisms, clinical features, and therapeutic strategies of PF in the context of IEI were summarized, aiming to enhance early recognition and targeted management of IEI complicated with PE in children.