Bronchodilator responsiveness in children with primary ciliary dyskinesia

Elias Seidl; Dvir Gatt; Wallace B Wee; David Wilson; Felix Ratjen; Hartmut Grasemann

doi:10.1183/23120541.00611-2023

Bronchodilator responsiveness in children with primary ciliary dyskinesia

ERJ Open Res. 2024 Jan 15;10(1):00611-2023. doi: 10.1183/23120541.00611-2023. eCollection 2024 Jan.

Authors

Elias Seidl^{1

2}, Dvir Gatt¹, Wallace B Wee¹, David Wilson¹, Felix Ratjen^{1

2}, Hartmut Grasemann^{1

3

4}

Affiliations

¹ Division of Respiratory Medicine, Department of Pediatrics, The Hospital for Sick Children, Toronto, ON, Canada.
² Division of Respiratory Medicine, University Children's Hospital Zurich, Zurich, Switzerland.
³ Translational Medicine Program, Research Institute, Hospital for Sick Children, Toronto, ON, Canada.
⁴ University of Toronto, Toronto, ON, Canada.

Abstract

Background: Reversible airway obstruction is common in children with primary ciliary dyskinesia. However, the diagnostic value of adding bronchodilator (BD) response testing to routine spirometry is unclear.

Methods: This is a retrospective analysis of pulmonary function test results obtained from children with primary ciliary dyskinesia seen as outpatients at the Hospital for Sick Children, Toronto. Spirometry results were collected for every appointment with BD response testing ("Visit", with pre-BD and post-BD measurements) as well as for the previous ("Baseline") and following ("Follow-up") encounters.

Results: A positive BD response was seen in 86 out of 474 (18.1%) of the pulmonary function tests from 82 children with primary ciliary dyskinesia. BD responsiveness was associated with a significant absolute change (±sd) in % predicted forced expiratory volume in 1 s (FEV₁) from Baseline to Visit pre-BD (-6.5±10.3%, p<0.001), but not from Baseline to Follow-up (0.4±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 FEV₁ % predicted compared to those with no BD response (-0.9% per year versus -0.5% per year, p<0.001). The annual decline in FEV₁ % predicted was greater in children with multiple compared to one measured positive BD responses (-1.3% per year versus -0.6% per 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 primary ciliary dyskinesia may help identify those at risk for accelerated lung disease progression.