PROGNOSIS - the German Bronchiectasis Registry: first results

Background: PROGNOSIS (www.bronchiektasen-register.de) is a representative registry, to adress important epidemiologic questions and to provide real-life data of current bronchiectasis management in Germany. Methods: Prospective, observational and longitudinal register study with baseline and follow—up data collection every year; recruitment across all levels of of healthcare. Results: Recruitment (8 Feb 2017, first 18 months): 706 subjects, 36 centers. Data from the first 505 validated datasets (50% academic centers, AC) will be presented. 250 validated datasets (66% AC) showed: average age 57±15 yrs (60% female; 54% never-smokers); median annual exacerbation 2 (0-3) and hospitalisation 0 (0-1) rate; ppFEV1 67±27%; FEV1/FVC ratio 0.67±0.16; BMI 24±5 kg/m2; 46% duration of bronchiectasis >10 years; radiological mainly basal distribution. Most common etiologies: 35% idiopathic, 11% COPD, 11% Primary Ciliary Dyskinesia, 10% postinfectious, 7% primary immunodeficiency, 7% asthma. Relevant pathogens if clinically stable: 37% P. aeruginosa (PA), 18% S. aureus , 13% A. fumigatus , 8% H. influenzae , 7% nontuberculous mycobacteria. 71% of PA positive subjects received at least one eradication attempt. Regular bronchiectasis therapy 90%: 78% physiotherapy, 64% mucoactive physio adjuncts, 33% azithromycin maintenance therapy, 26% inhaled antibiotics, 12% prior thoracic surgery. Vaccination coverage: pneumococcal PPSV23 50% and PCV13 38%, influenza 79%. Pulmonary rehab 44%. Conclusions: Data structure is likely to change due to more non-academic centers. Basic treatments (e.g. vaccination) are underused. Interventional clinical trials are needed. Support: CAPNETZ STIFTUNG, BREATH/DZL, Bayer, Grifols, Insmed, IMI (EU/EFPIA).