Airway disease and inhaled corticosteroids (ICS) in children with primary ciliary dyskinesia (PCD)

Abstract

PCD is characterized by neutrophilic airways disease, but despite the fact that many patients are prescribed ICS, the contribution of Type 2 inflammation is often not well characterized. We hypothesized that ICS are overprescribed in PCD.

We studied 103 children (46 girls) with confirmed PCD (age 9.9±4.4 years). Forty-eight patients (46.6%) were atopic based on elevated total IgE and/or specific IgE to common inhalant allergens. Thirty-eight patients (37%) were prescribed ICS. Patients who were and were not treated with ICS did not differ significantly in FEV₁ (median [interquartile range] 82 [70.5-87.5] vs 85 [80-92] % pred), atopy (50% vs 43%), vitamin D levels (47 [32-57] vs 53 [34-66] nmol/l), extent of chest CT changes, and fractional exhaled nitric oxide (FeNO, 5 [5-8.‘5‘] vs 7.5 [5.3-12.5] pbb). Bronchodilator reversibility (BDR) >10% was found in 28% but was not associated with atopy (OR 2.13, 95%, 95% CI 0.5-8.5), FeNO (OR 0.9, 95%CI 0.1-7.7), or being treated with ICS (OR 2.1 95% CI 0.54-8.2). Those prescribed ICS showed slightly greater BDR (8 [6-14] vs 4 [1-10] %). Eosinophilia in broncho-alveolar lavage was rare (4/32), and almost exclusively seen in atopic patients. When comparing atopic and non-atopic PCD patients, atopic patients had significantly higher FeNO (median 10.5 [7.5-13] vs ‘5 [5-6.5] ‘ppb, p=0.008), but values above normal were not seen.

We conclude that atopy and BDR are common in children with PCD. ICS do not seem to be well targeted to children with Type 2 inflammation, FeNO is never above normal, implying sputum eosinophilia should be measured before prescribing ICS. Acute BDR of itself is not a sufficient reason for prescribing ICS.