Association of German Bronchiectasis Registry Participation With Disease Course

Background Bronchiectasis research has advanced significantly through international and national registries, such as the European bronchiectasis registry (EMBARC) revealing crucial insights into the disease's heterogeneous nature. These efforts underscore the importance of precise patient phenotyping and the identification of underlying causes to improve management and outcomes in bronchiectasis. Study design and methods The prospective, non-interventional PROGNOSIS registry has collected data from over 1500 CT-confirmed adult bronchiectasis patients across 38 German sites, excluding those with cystic fibrosis or lung transplants. Aligned with the EMBARC registry, it ensures comprehensive clinical data collection. Statistical analyses of baseline and follow-up data aim to assess disease progression and patient outcomes. Results Key outcomes include a reduction in the median exacerbations (IQR, 1(1–4) to 1 (1–3), p<0.001) and hospitalizations (IQR, 1(1–2) to 1(1–1), p<0.001) demonstrating an improvement in managing the disease during patients’ participation in the registry. Lung function (ppFEV1, IQR 73%(51–90) to 73%(52–92), p=0.6) remained stable over time, contradicting the expected decline in such chronic lung conditions. There was a significant decrease in active smokers (4.4% to 3.6%, p<0.001), and improvements were seen in sputum quantity and quality. There was an increase in the detection of pathogens, particularly Pseudomonas aeruginosa (32% to 36%, p<0.001). Notable shifts in the underlying causes of bronchiectasis were observed over the registry period, including a decrease in idiopathic cases (35% to 28%, p<0.001) and an increase in cases with a proven etiology. Conclusion The PROGNOSIS registry highlights the critical role of comprehensive management in bronchiectasis, emphasising infection control and treatment adherence. It underscores the need for personalised treatment by identifying the underlying aetiology and reinforces the ongoing importance of research in improving patient care and quality of life.