Pulmonary fibrosis (PF) in children is a rare complication of specific forms of childhood interstitial lung diseases (chILD) with extremely limited scientific evidence to guide optimal management. Whilst there continues to be significant progress in PF management for adult populations, paediatric guidelines have stagnated. New anti-fibrotic medications (nintedanib and pirfenidone) are finding regular use amongst adult PF patients but remain largely unstudied and untested in children. Although there are major differences between the two age-group populations, it is useful to learn from the evolution of adult PF management, especially in the absence of dedicated paediatric studies. Whilst there have been recent trials aimed at assessing the safety and efficacy of drugs such as nintedanib and hydroxychloroquine, there is still a dire need for more research aimed at further assessing current treatment practices and evaluating the safety and efficacy of new emerging treatments in the paediatric population.

儿童肺纤维化（PF）是特定形式的儿童间质性肺疾病（chILD）的罕见并发症，指导最佳治疗的科学证据极其有限。尽管成人肺纤维化管理继续取得重大进展，但儿科指南却停滞不前。新的抗纤维化药物（尼达尼布和吡非尼酮）在成人肺纤维化患者中经常使用，但在儿童中仍然很大程度上未经研究和测试。尽管两个年龄组人群之间存在重大差异，但了解成人 PF 管理的演变是有用的，特别是在缺乏专门的儿科研究的情况下。虽然最近进行了旨在评估尼达尼布和羟氯喹等药物的安全性和有效性的试验，但仍然迫切需要进行更多研究，以进一步评估当前的治疗实践并评估儿科新兴治疗方法的安全性和有效性人口。