The german bronchiectasis registry (PROGNOSIS): results from 1000 patients

Background The objective of the German Bronchiectasis Registry PROGNOSIS is to set-up a representative registry, which provides answers to the most important epidemiologic questions as well as real-life data regarding the current management of bronchiectasis across different levels of healthcare in Germany.

Methods Prospective, observational and longitudinal register study with baseline and follow-up data collection every year; recruitment across all levels of healthcare.

Results During the first 36 months 1155 patients were recruited in 38 centers per 24 September 2018. Analysis of the first 1000 validated datasets showed an average age of 59 ± 16 yrs (59% female; 55% never-smokers) and median annual exacerbation and hospitalization rates of 1 (0 – 3) and 0 (0 – 1), respectively. On average moderate airflow obstruction was present (FEV1 73 ± 25% pred.). The duration of bronchiectasis was > 10 years in 41% of subjects. The 5 most common etiologies were: idiopathic (36%), postinfectious/post-TB (21%), COPD (15%), asthma (11%) and Kartagener syndrome/Primary Ciliary Dyskinesia (PCD; 9%). The most common relevant pathogens if clinically stable were Pseudomonas aeruginosa (PA; 38%), Staphylococcus aureus (16%) and Haemophilus influenzae (12%). Overall 60% of subjects practiced routine respiratory physiotherapy, while 77% of subjects received regular pharmacologic treatment for bronchiectasis: mucoactive physiotherapy adjuncts (84%), short-acting bronchodilators (77%), long-acting muscarinic agents (50%), inhaled corticosteroid/long-acting bronchodilator combinations (38%), long-acting betaadrenergic agents (32%), azithromycin maintenance therapy (29%) and inhaled antibiotics (29%) were most commonly prescribed.

Conclusions PA is the most relevant pathogen and is associated with disease severity. Basic treatments like routine respiratory physiotherapy, vaccinations and pulmonary rehabilitation are considerably underused.