The prognosis of people with cystic fibrosis (pwCF) has improved dramatically with the introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulators (CFTRm). The ageing of the cystic fibrosis (CF) population is changing the disease landscape with the emergence of different needs and increasing comorbidities related to both age and long-term exposure to multiple treatments including CFTRm. Although the number of pwCF eligible for this treatment is expected to increase, major disparities in care and outcomes still exist in this population.
View Article and Find Full Text PDFPaediatric and adult bronchiectasis patients have been addressed in the literature as two different populations due to several differences, but there is insufficient evidence to understand how and when disease characteristics really change along patients' lifespan. This lack of knowledge is evident in all aspects of the transition: insufficient data is available about radiology, lung function, microbiology and treatment, and only limited information is currently available about changes in clinical presentation and psychosocial aspects. For instance, symptoms seem to improve during the third and fourth decades of life, a period sometimes referred to as the "honeymoon phase".
View Article and Find Full Text PDFBackground: Asthma is commonly reported in patients with a diagnosis of bronchiectasis.
Objective: The aim of this study was to evaluate whether patients with bronchiectasis and asthma (BE+A) had a different clinical phenotype and different outcomes compared with patients with bronchiectasis without concomitant asthma.
Methods: A prospective observational pan-European registry (European Multicentre Bronchiectasis Audit and Research Collaboration) enrolled patients across 28 countries.