YOUNG adults with bronchiectasis that begins in childhood face significantly more severe disease in adulthood compared to those with adult-onset bronchiectasis, according to new data from the European Bronchiectasis Registry (EMBARC).
Researchers analysed data from 1,422 patients across four EMBARC centres, comparing 249 individuals with paediatric-onset bronchiectasis (POBE), defined as symptom onset before age 18 years, to 1,173 with adult-onset bronchiectasis (AOBE). POBE patients had a mean age of onset of 6.5 years, while patients with AOBE developed symptoms at an average age of 55.4 years. The study aimed to clarify the characteristics, severity, and potential risk factors contributing to disease burden in the under-recognised POBE subgroup.
The results demonstrated that patients with POBE had more severe clinical and radiological disease features despite being younger at enrolment (mean age 50.3 vs. 66 years). They had significantly worse lung function (mean FEV₁% predicted: 70.8% vs. 84.2%), higher radiological severity (mean Reiff score: 6.0 vs. 4.4), and a greater prevalence of chronic bacterial infection (72.3% vs. 54.6%). Patients with POBE also reported more frequent annual exacerbations (median: 2 vs. 1) and longer symptom duration (43.3 vs. 10.8 years). All comparisons were statistically significant (p<0.001).
Multivariable models revealed that longer symptom duration was independently associated with poorer outcomes, including Pseudomonas aeruginosa infection, increased hospitalisations, more frequent exacerbations, and further reductions in lung function. The presence of congenital causes, such as primary ciliary dyskinesia and primary immunodeficiencies, was more common in the POBE group and contributed to disease severity. Notably, existing bronchiectasis severity scoring tools may underestimate risk in this population due to their younger age and longer disease course.
The authors suggest that POBE should be recognised as a distinct clinical entity requiring tailored care pathways, including earlier and more intensive management and better structured transition from paediatric to adult respiratory services.
Reference
Khalaili L et al. Greater disease severity in adults with paediatric-onset versus adult-onset bronchiectasis: a multicenter EMBARC registry study. Eur Respir J. 2025; DOI: 10.1183/13993003.00665-2025.
