Lung Function in Pediatric Non-cystic Fibrosis Bronchiectasis Patients Compared to Cystic Fibrosis Patients: A 20-year Retrospective Cohort Study

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Abstract Rationale: Pediatric non-cystic fibrosis bronchiectasis (NCFB) is a heterogenous pulmonary disorder, characterized by chronic mucosal inflammation, recurrent infection, airway remodeling, and impaired mucociliary clearance. These factors contribute to a significant symptomatic burden in affected children. Despite the growing recognition of NCFB, there remains a limited understanding of its clinical outcomes, largely due to the scarcity of dedicated studies focused on pediatric cohorts. Most current understandings is based on comparisons with pediatric cystic fibrosis (CF) and adult NCFB populations. This study aims to characterize pulmonary function in a large pediatric NCFB cohort. Methods: We conducted a retrospective cohort study using clinical data obtained from Boston Children's Hospital Pulmonary Function Lab from 2004-2023. We identified children with NCFB by at least one ICD9 or ICD10 code for bronchiectasis, excluding CF codes, and at least two pulmonary function tests (PFTs) before age 18 with a minimum one-year interval between their first and last lung function test. We constructed a similar cohort of patients with CF ICD9 and ICD10 codes for comparison. We collected data on age, sex, spirometry visits (FEV1, FVC, FEV1/FVC), the number of spirometry visits, and the years over which spirometry was obtained. We estimated percent predicted values for FEV1 and FVC using Global Lung Initiative (GLI) reference values. We performed non-parametric tests to compare age, number of tests and follow-up, and lung function. We performed Fisher's Exact Test to compare sex. Results: We identified a total of 302 NCFB and 672 CF pediatric patients who also obtained spirometry over at least 1 year (Table 1). When compared to the CF group, NCFB patients were older at first spirometry (median age 10.2 vs 8.7 years) more likely to be female (58% vs 46%), had shorter length of follow-up (median years 4.6 vs 8.8 years) and had fewer spirometry visits per year (median 2.0 vs 3.5). Initial FEV1 and FVC percent predicted were also significantly lower in NCFB patients compared to the CF cohort (all p<0.001). Conclusions: In our study of pediatric NCFB and CF patients, those with NFCB were older, predominantly female, and exhibited lower lung function at their first spirometry test. Further work is needed to address specific biases present in this clinical cohort data, as well as to construct models for lung function change and prognosis. Our work further underscores the need for ongoing research in pediatric NCFB particularly to understand disease progression and outcomes.

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