European IPF/ILD Registry & Biobank (eurIPFreg/Bank, eurILDreg/Bank), Giessen, Germany.
Center for Interstitial and Rare Lung Diseases, Universities of Giessen and Marburg Lung Center (UGMLC), Justus-Liebig-University Giessen, Member of the German Center for Lung Research (DZL), Giessen, Germany.
BMC Pulm Med. 2024 Nov 18;24(1):572. doi: 10.1186/s12890-024-03389-9.
Interstitial lung diseases (ILDs), encompassing both pediatric and adult cases, present a diverse spectrum of chronic conditions with variable prognosis. Despite limited therapeutic options beyond antifibrotic drugs and immunosuppressants, accurate diagnosis is challenging, often necessitating invasive procedures that may not be feasible for certain patients. Drawn against this background, experts across pediatric and adult ILD fields have joined forces in the RARE-ILD initiative to pioneer novel non-invasive diagnostic algorithms and biomarkers. Collaborating with the RARE-ILD consortium, the eurILDreg aims to comprehensively describe different ILDs, analyze genetically defined forms across age groups, create innovative diagnostic and therapeutic biomarkers, and employ artificial intelligence for data analysis.
The foundation of eurILDreg is built on a comprehensive parameter list developed and adopted by clinical experts, encompassing over 1,800 distinct parameters related to patient history, clinical examinations, diagnosis, lung function and biospecimen collection. This robust dataset is further enriched with daily assessments captured through the patientMpower app, including handheld spirometry and exercise tests, conducted on approximately 350 patients over the course of a year. This approach involves app-based daily assessments of quality of life, symptom tracking, handheld spirometry, saturation measurement, and the 1-min sit-to-stand test (1-STST). Additionally, pediatric data from the ChILD-EU registry will be integrated into the RARE-ILD Data Warehouse, with the ultimate goal of including a total of 4.000 ILD patients and over 100.000 biospecimen.
The collaborative efforts within the consortium are poised to streamline research endeavors significantly, promising to advance patient-centered care, foster innovation, and shape the future landscape of interstitial lung disease research and healthcare practices.
EurILDreg is registered in the German Clinical Trials Register (DRKS 00028968, 26.07.2022), and eurIPFreg is registered in ClinicalTrials.gov (NCT02951416).
间质性肺疾病（ILDs），包括儿科和成人病例，呈现出多种慢性疾病，预后各不相同。尽管除了抗纤维化药物和免疫抑制剂之外，治疗选择有限，但准确诊断具有挑战性，通常需要进行侵入性程序，但某些患者可能无法进行。在这种背景下，儿科和成人ILD 领域的专家们在 RARE-ILD 倡议中联手，开创了新的非侵入性诊断算法和生物标志物。eurILDreg 与 RARE-ILD 联盟合作，旨在全面描述不同的 ILD，分析不同年龄段遗传定义的形式，创建创新的诊断和治疗生物标志物，并使用人工智能进行数据分析。
eurILDreg 的基础是建立在临床专家制定和采用的全面参数列表上，该列表包含超过 1800 个与患者病史、临床检查、诊断、肺功能和生物样本采集相关的独特参数。这个强大的数据集通过 patientMpower 应用程序进行了进一步丰富，该应用程序每天捕获大约 350 名患者的日常评估，包括手持式肺活量测定和运动测试。这种方法涉及基于应用程序的日常评估，包括生活质量、症状跟踪、手持式肺活量测定、饱和度测量和 1 分钟坐立测试（1-STST）。此外，来自 ChILD-EU 登记处的儿科数据将被整合到 RARE-ILD 数据仓库中，最终目标是纳入总共 4000 名 ILD 患者和超过 100000 个生物样本。
联盟内的合作努力有望显著简化研究工作，有望推进以患者为中心的护理，促进创新，并塑造间质性肺疾病研究和医疗保健实践的未来格局。
eurILDreg 在德国临床试验注册处（DRKS 00028968，2022 年 7 月 26 日）注册，eurIPFreg 在 ClinicalTrials.gov 注册（NCT02951416）。
