TY - JOUR T1 - Airway disease and inhaled corticosteroids (ICS) in children with primary ciliary dyskinesia (PCD) JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2016.PA1601 VL - 48 IS - suppl 60 SP - PA1601 AU - Eleonora Dehlink AU - Charlotte Richardson AU - Gemma Marsh AU - Kiri Pares AU - Angela Jamalzadeh AU - Andy Bush AU - Claire Hogg AU - Siobhán B. Carr Y1 - 2016/09/01 UR - http://erj.ersjournals.com/content/48/suppl_60/PA1601.abstract N2 - 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. ER -