Raising diagnostic accuracy and therapeutic perspectives in interstitial lung diseases

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Pediatric and adult Interstitial Lung Diseases (ILD) are a heterogenous group of >100 different, rare diseases, which share the fate of progressivescarring and, ultimately, death. Apart from two novel anti-fibrotic drugs authorized only for IPF and steroids/immunosuppressants used forinflammatory-driven ILDs, therapeutic options are scarce and lung transplantation represents the only curative option. Similarly, settling a correctdiagnosis is difficult in most cases, especially when patients are too sick to undergo invasive procedures. Drawn against this background, leadingexperts in their fields joined this initiative to i) develop novel, primarily non-invasive, diagnostic algorithms and biomarkers, to ii) better understand thenatural course and the role of intermittent exacerbations, to iii) develop better prognostic models and biomarkers, to iv) better understand genotype /phenotype relationships and v) to a priori predict response to therapy via novel therapeutic biomarkers. Based on already existing pediatric (chILD-EU)and adult (eurIPFreg) registries / biobanks, we aim to include another 5000 ILD patients and > 100.000 biomaterials. Next to deep phenotyping, weintend to conduct daily hand-held spirometry, accelerometry and saturation measurement, periodic capture of volatile organic solvent (VOC) basedsignatures (via awarded “Sniffphone”), apply proteomics/genomics in exhaled breath condensates (EBC)s and deep sequencing in EDTA blood, useartificial intelligence for CT interpretation and, finally, establish disease models on the basis of big data analyses.