David Gozal, Martin Kolb
European Respiratory Journal 2023 61: 2201797; DOI: 10.1183/13993003.01797-2022

Extract

Childhood interstitial lung disease (chILD) describes a group of rare lung diseases that can affect infants, children and adolescents, and is associated with a high morbidity and mortality [1]. Typical features of chILD include dyspnoea, the presence of diffuse infiltrates on chest radiographs and abnormal pulmonary function tests with primarily evidence of a restrictive ventilatory defect along with impaired gas exchange. Due to the diversity of conditions ultimately resulting in this clinical phenotype, chILD is difficult to diagnose, and the classification scheme is frequently problematic even in the most specialised centres. This has fostered the formation of multinational collaborative networks [2]. Notwithstanding, despite substantial diversity in the upstream pathophysiological pathways involved in the chILD spectrum of conditions, a subset of these children will develop progressive fibrosing lung disease, which ultimately is fraught with adverse outcomes. The emergence in recent years of multiple drugs targeting the cessation or even reversal of fibrosis in the context of adult ILD prompted a call to arms advocating the judicious use of such agents in children [3].