Old and emerging therapies for childhood interstitial lung disease (chILD): a systematic review

Childhood interstitial lung disease (chILD) is a heterogeneous group of rare lung diseases that comprises more than 200 entities. The diagnostic process for chILD might require multiple investigations, which often include a combination of clinical assessments, imaging studies, genetic testing and invasive procedures. Given the prolonged diagnostic timeline, supportive treatments are typically initiated followed by more targeted therapies generally postponed until the underlying cause of the disease is identified. This systematic review aims to describe the current knowledge regarding different treatment strategies for chILD and critically appraise, compare and qualitatively summarise the current evidence on old and emerging therapies for chILD. Of the 5775 publications returned from the initial search, 100 studies met the inclusion criteria, of which 50% were reviews or task force reports from several scientific societies, 41% case reports or case series, 5% randomised controlled trials and 4% preclinical studies on emerging therapies. In order to provide a clearer description of the data analysed, we describe available therapeutic options for chILD in general, organised by treatment type, and then we report treatments categorised by specific pathology.