European network for translational research in children's and adult interstitial lung disease

Financed by : European Commission


Reference: CA16125

Duration: April 2017 to July 2021



Interstitial lung disease in children (chILD) is a term that describes a collection of more than 200 rare lung disorders. It is a heterogeneous group of non-neoplastic disorders resulting from damage by varying patterns of inflammation and fibrosis with the interstitium as the primary site of injury. As with other orphan diseases, chILD data is lacking on the natural course, phenotypic variability, associations with genotype, and effectiveness of treatments. The disease course is very variable, and depending on more than just the underlying cause; for example, within a given family, the phenotypic variability of chILD such as surfactant protein C mutation is huge. The rarity of individual chILDs contributes to a lack of randomised control trial data on effectiveness of treatments. Management strategies derive from other diseases or are based on physicians experience and remain controversial.

This Action will create a pan-Europe-led network of multidisciplinary clinicians (adult and paediatric), scientists, and patients and their families with the aim of accurate and early diagnosis with structured, potentially personalised, management and therapies. The Action will stimulate and coordinate multidisciplinary research in chILD from infancy to adulthood, as well as reveal the pathophysiological commonalities between different forms ILD at the molecular level. The results of these efforts will create large incremental changes in understanding and management of chILD. Since chILD is an umbrella term for a number of conditions most of which imply more than purely medical or scientific expertise, the Action will pay due attention to the larger societal implications of chILD research

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