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Project Topic
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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 progressive scarring and, ultimately, death. Apart from two novel anti-fibrotic drugs authorized only for IPF and steroids/immunosuppressants used for inflammatory-driven ILDs, therapeutic options are scarce and lung transplantation represents the only curative option. Similarly, settling a correct diagnosis is difficult in most cases, especially when patients are too sick to undergo invasive procedures. Drawn against this background, leading experts in their fields joined this initiative to i) develop novel, primarily non-invasive, diagnostic algorithms and biomarkers, to ii) better understand the natural 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, we intend to conduct daily hand-held spirometry, accelerometry and saturation measurement, periodic capture of volatile organic solvent (VOC) based signatures (via awarded “Sniffphone”), apply proteomics/genomics in exhaled breath condensates (EBC)s and deep sequencing in EDTA blood, use artificial intelligence for CT interpretation and, finally, establish disease models on the basis of big data analyses.
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