C61-20 Non Cystic Fibrosis Bronchiectasis in Children: Correlation Between Clinical Presentation and Radiological Score

Rationale

Pediatric non-cystic fibrosis (non-CF) bronchiectasis is a heterogeneous and often underdiagnosed condition in which radiological severity does not always reflect clinical or functional burden. Semi-quantitative imaging tools such as the modified Reiff score may help integrate structural assessment with clinical and functional parameters. Our aim was to evaluate the association between radiological severity of pediatric non-CF bronchiectasis, assessed by the modified Reiff score with clinical burden and pulmonary function.

Methods

We conducted a cross-sectional study including patients aged <18 years with non CF-bronchiectasis enrolled from January 2025 to January 2026 at “Sapienza” University of Rome. The modified Reiff score was independently calculated by three operators with consensus agreement. Pulmonary function tests were performed according to ATS/ERS standards in compliants patients. Associations were assessed using Pearson’s correlation coefficient. Pulmonary exacerbation were defined according to the last ERS guidelines on management of pediatric non-CF bronchiectasis. A p < 0.05 was considered statisticallt significant.

Results

Thirty-one patients were included (18 males, median age 9.41±3.83, age range: 2-18 years). Regarding etiology, the most frequent cause of non-cystic fibrosis bronchiectasis was idiopathic (25.8%), followed by primary ciliary dyskinesia (19.4%), post-infectious disease (19.4%), and chronic aspiration (9.7%). The remaining cases (25.4%) were equally distributed among tracheoesophageal fistula, bronchiolitis obliterans, genetic syndromes, and congenital lung malformations. Twenty-five patients underwent spirometry. The median modified Reiff score was 2 (range 1-10). The modified Reiff score was inversely correlated with FEV1% predicted (p = 0.025), FEV1 z-score (p = 0.044) and BMI z-score (p = 0.038). No significant associations were observed between the modified Reiff score and exacerbations in the last 6-12 months or hospitalization frequency in the previous year.

Conclusions

In pediatric non-CF bronchiectasis, greater radiological severity assessed by the modified Reiff score is associated with worse pulmonary function and poorer nutritional status, but not with recent clinical burden. These findings suggest that the modified Reiff score primarily reflects cumulative structural damage and its functional impact. Incorporating standardized radiological scoring alongside pulmonary function and nutritional assessment may enhance disease characterization and longitudinal monitoring in pediatric non-CF bronchiectasis.

This abstract is funded by: Sapienza University (Bando Avvio alla Ricerca”