European Respiratory Journal最新文献

Background: Young adults with paediatric-onset bronchiectasis (POBE) represent a minority of bronchiectasis patients and are poorly characterised. We aimed to compare the characteristics and severity of adults with POBE to adult-onset bronchiectasis (AOBE).

Methods: Data from four EMBARC (European Multicentre Bronchiectasis Audit and Research Collaboration) centres were analysed. POBE was defined as patient-reported symptom onset before the age of 18 years, while AOBE was defined as symptom onset at or after the age of 18 years. We compared POBE to AOBE and used multivariable models to identify factors associated with disease severity, including lung function, Pseudomonas aeruginosa infection, exacerbations and hospitalisations.

Results: Among 1422 patients, 249 (17.5%) had POBE (mean onset age: 6.5 years) and 1173 had AOBE (mean onset age: 55.4 years). POBE patients were younger at enrolment (50.3 versus 66 years), had longer disease duration (43.3 versus 10.8 years), worse lung function (forced expiratory volume in 1 s (FEV₁): 70.8% versus 84.2% predicted), greater radiological extent (Reiff score: 6.0 versus 4.4), higher bacterial infection rates (72.3% versus 54.6%) and more exacerbations (median: 2 versus 1 per year) compared to AOBE (p<0.001 across all comparisons). Symptom duration was independently associated with P. aeruginosa infection, hospitalisation, exacerbations and reduced FEV₁ % pred. Congenital aetiologies, such as primary ciliary dyskinesia and primary immunodeficiencies, further contributed to disease severity.

Conclusions: Adults with POBE exhibit greater disease severity than those with AOBE, likely due to prolonged symptom duration and congenital aetiologies. Conventional bronchiectasis severity scores may underestimate severity in young people with POBE. Optimised care, including structured transition to adult care, may mitigate progression in POBE patients.

Background: The growing popularity of cannabis smoking in an era of legalisation has prompted concerns about respiratory health.

Objective: To investigate clinical and airway epithelial transcriptomic features associated with cannabis smoking.

Methods: This cross-sectional study analysed data from 139 cannabis-smoking participants categorised by joint-year exposure (low: ≤5; moderate: >5-20; high: >20 joint-years) and 57 never-smokers. We evaluated respiratory symptom questionnaire scores, lung function measurements, chest computed tomography and hyperpolarised ¹²⁹Xenon pulmonary magnetic resonance imaging measurements across groups. We compared the expression of immune response signatures and mucin genes in airway epithelial brushings collected from bronchoscopy. Using air-liquid interface cell cultures, we quantified epithelial mucin 5AC (MUC5AC) protein and correlated its expression with clinical outcomes.

Results: Among cannabis-smoking participants (48% male, median age of 27 years), 84% reported current or former cigarette smoking or vaping. Cannabis-smoking groups reported worse respiratory symptoms than never-smokers. High joint-year cannabis-smoking participants showed lower pre-bronchodilator forced expiratory volume in 1 s to forced vital capacity ratio, lower forced expiratory flow at 25-75% of the forced vital capacity, more radiographic emphysema and more ventilation abnormalities than never-smokers. Airway epithelial brushings from cannabis-smoking participants demonstrated an increased type 2 immune response, decreased type 17 immune response and higher MUC5AC gene expression than non-cannabis-smoking participants. Epithelial MUC5AC protein expression in cell cultures correlated with worse clinical outcomes and imaging abnormalities.

Conclusions: Cannabis smoking, particularly at high exposures, is associated with worse respiratory symptoms, lower lung function, functional imaging abnormalities and dysregulated immune responses in the airway epithelium. These observations suggest respiratory harm associated with cannabis smoking and underscore the concerns for future respiratory morbidities related to persistent cannabis use.