Abstract
Background Reversible airways obstruction has been reported to be common in people with primary ciliary dyskinesia (PCD). However, the diagnostic value of bronchodilator (BD) response testing added to routine spirometry is unclear.
Methods This is a retrospective analysis of pulmonary function test (PFT) results obtained from children with PCD seen as outpatients at the Hospital for Sick Children, Toronto. Spirometry results for every Visit with BD-response testing, the previous (Baseline) and the following (Follow-up) encounters were collected.
Results A positive BD-response was seen in 86/474 (18.1%) of PFTs from 82 children with PCD. BD-responsiveness was associated with a significant absolute change in percent predicted forced expiratory volume in one second (ppFEV1) from Baseline to Visit pre-BD ppFEV1 (−6.5%, sd 10.3, p<0.001), but not from Baseline to Follow-up (0.4%, sd 10.8, p=0.757). Antimicrobial therapy was initiated more commonly following a Visit with a positive BD-response (OR 3.8, 95%CI 2.2–6.6) compared to no BD-response. Children with a positive BD-response had a greater annual decline in ppFEV1 compared to those with no BD-response (−0.9%/year versus −0.5%/year; p<0.001). The annual decline in ppFEV1 was greater in children with multiple compared to one measured positive BD responses (−1.3/year versus −0.6/year, p<0.001) and in those not treated with antibiotic therapy following a positive BD response compared to those treated with antibiotics (−1.1% versus −0.6%; p<0.001).
Conclusion A positive BD-response in children with PCD may help identify those at risk for accelerated lung disease progression.
Footnotes
This manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.
Conflict of Interest: There are no competing interests for any author.
This is a PDF-only article. Please click on the PDF link above to read it.
- Received August 22, 2023.
- Accepted November 5, 2023.
- Copyright ©The authors 2023
This version is distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0. For commercial reproduction rights and permissions contact permissions{at}ersnet.org
