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].
Abstract
Interstitial lung disease in children lacks therapeutic options: as reported by a first RCT of its kind, a subset of these children presenting with a fibrotic phenotype may benefit from a therapeutic trial with nintedanib https://bit.ly/3fuTYaW
Footnotes
Conflict of interest: D. Gozal has no conflicts of interest to disclose. M. Kolb reports being site PI for sponsored clinical trials for Roche and Boehringer Ingelheim, grants from Canadian Institute for Health Research, grants and personal fees for advisory board work from Boehringer Ingelheim and Roche Canada, chief editor allowance from the European Respiratory Society, personal fees for advisory board work from Horizon, Algernon, CSL Behring, DevPro, Bellerophon and BMS, personal fees for adjudication committee work from United Therapeutics, and personal fees for data monitoring committee work from LabCorp, outside the submitted work.
- Received September 14, 2022.
- Accepted September 20, 2022.
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