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. 2022 Nov 16;11(22):6791.
doi: 10.3390/jcm11226791.

Reversible Bronchial Obstruction in Primary Ciliary Dyskinesia

Hagit Levine  1   2 , Ophir Bar-On  1   2 , Vered Nir  3 , Nicole West  2 , Yotam Dizitzer  4 , Huda Mussaffi  1   2 , Dario Prais  1   2
Affiliations

Reversible Bronchial Obstruction in Primary Ciliary Dyskinesia

Hagit Levine et al. J Clin Med. .

Abstract

Background: Inhaled bronchodilators are frequently used among patients with primary ciliary dyskinesia (PCD), although neither the effectiveness nor the prevalence of their use is known, due to the paucity of relevant studies.

Methods: This is a retrospective analysis of pre- and post-bronchodilator spirometry results, of patients with PCD from two centers. Correlations were examined of bronchodilator response, with asthma and atopy markers.

Results: Of 115 patients, 46 (40%) completed spirometry pre- and post-bronchodilation. Of these, 26 (56.5%) demonstrated reversible airway obstruction (increase in %FEV1 predicted ≥ 10%). Obstruction reversibility was not found to be associated with a family history of asthma, blood eosinophil level, elevated IgE, or atopy symptoms. Of the 46 patients who completed bronchodilator spirometry, 29 (63%) were regularly using bronchodilators and inhaled corticosteroids.

Conclusions: More than half of patients with PCD presented with reversible airway obstruction, without any correlation to markers of personal or familial atopy. Inhaled bronchodilators and corticosteroid therapies are commonly used for treating PCD. Evaluating bronchodilator response should be considered, and its effectiveness should be further studied.

Keywords: airway hyperreactivity; airway obstruction; asthma; bronchodilators; inhaled corticosteroids; primary ciliary dyskinesia; spirometry.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
%FEV1 predicted for age comparing patient with primary ciliary dyskinesia with or without airway reversibility.
Figure 2
Figure 2
Inhaled bronchodilator and corticosteroid usage among patients with primary ciliary dyskinesia.
See this image and copyright information in PMC

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