Raising diagnostic accuracy and therapeutic perspectives in interstitial lung diseases

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.

小儿和成人间质性肺部疾病（ILD）是一组> 100种不同的罕见疾病的异源群，它们具有进步性疾病的命运，最终是死亡。除了仅授权IPF和用于炎症驱动的ILD的类固醇/免疫抑制剂的两种新型抗纤维化药物外，治疗选择是稀缺的，肺移植代表了唯一的治疗选择。同样，在大多数情况下，设置正确的诊断很困难，尤其是当患者病得无法接受侵入性手术时。在这种背景下，领域的领导者加入了这一举措，以i）开发新型的，主要的非侵入性，诊断算法和生物标志物，ii）更好地理解间歇性加剧的自然过程和作用，to III的iii），iii）通过更好地预测预后模型和生物标记，以更好地预测孔疗法，以更好地预测型号，以更好地预测基因类型和v），从生物标志物。基于已经存在的儿科（儿童欧盟）和成人（Euripfreg）注册机构 /生物库，我们的目标是包括另外5000名ILD患者和> 100.000个生物材料。在深度表型的旁边，Weintend要进行日常手持式肺活量法，加速度测量和饱和度测量，定期捕获基于挥发性的有机溶液（VOC）签名（通过授予“嗅探”）（通过授予的“嗅探”），将蛋白质组学/基因组应用于耗尽的呼吸凝结（EBC）中，并在Edta的呼吸模型中，最终在Edta的疾病中，最终建立了COT ARTICTAINS，并在Edta的血液中建立了COT，COT contection of Matlessions contemation in USEART SIMITACT，CT分析。