Plain language summary
- Childhood interstitial lung disease (chILD) describes a group of rare lung diseases affecting infants, children and adolescents.
- Some patients with chILD develop fibrosis (scarring) of the lungs, which progresses (worsens) over time.
- The InPedILD trial investigated the effects of a drug called nintedanib, which is used to treat lung fibrosis in adults, in children and adolescents aged 6-17 years with lung fibrosis due to chILD. The patients were randomly allocated to receive nintedanib or placebo for 24 weeks. The dose that the patients received was decided based on their weight.
- The results showed that the level of nintedanib in the blood of the children and adolescents in this trial was within the range in adults treated with the recommended dose.
- The side-effects of nintedanib in children and adolescents were similar to those seen in adults.
- Changes in lung function and oxygen saturation (the amount of oxygen carried by the blood) during the trial suggested that there may be benefits of nintedanib, but more data are needed.
- Together with what is known about the effects of nintedanib in adults with lung fibrosis, the results of the InPedILD trial provide further evidence to support the use of nintedanib in children and adolescents with lung fibrosis due to chILD.