TY - JOUR T1 - Differences in airway inflammation between children with primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) JF - European Respiratory Journal JO - Eur Respir J VL - 44 IS - Suppl 58 SP - P1207 AU - Hartmut Grasemann AU - Valerie Waters AU - Nancy McDonald AU - Yvonne Yau AU - Sharon Dell AU - Felix Ratjen Y1 - 2014/09/01 UR - http://erj.ersjournals.com/content/44/Suppl_58/P1207.abstract N2 - PCD and CF lung disease are similar in many aspects, but may differ in inflammatory responses. We compared markers of airway inflammation in patients with mild PCD or CF pulmonary exacerbation.Methods: Patients with an established diagnosis of CF or PCD were recruited from clinic if able to do pulmonary function testing and produce sputum. P. aeruginosa or B. cepacia complex infections were among the exclusion criteria. Sputum was obtained before (V1) and after (V2) 21 days of oral antibiotic treatment and analyzed for qualitative and quantitative micobiology, cell counts, interleukin-8 (IL-8) concentrations and neutrophil elastase activity.Results: Eleven PCD and 16 CF patients were included. Mean age was 10 (range 7-16) yrs in both groups. Mean (±SD) FEV1 at V1 was 63 (±13.8) % in PCD and 74 (±18.3) % predicted in CF (p=0.1). FEV1 improved to 71 (±21.2) % in PCD (p<0.05, paired t-test) and 86 (±12.3) % predicted in CF (p<0.01) on V2. Combined S. aureus and H. influenza density in sputum at V1 was significantly lower in PCD than CF. Sputum cell counts were not different. Mean (±SEM) IL-8 levels in sputum were similar in PCD and CF on V1 (237.3±77.8 vs 118.4 ±27.7 ng/ml, p=0.09) and V2 (211.5±105.2 vs 138.4±35.0 ng/ml, p=0.9). In contrast, while sputum elastase activity was similar in PCD and CF at V1 (83.0 ±15.0 vs 61.5±14.8 nanomol/min, p=0.16) it decreased at V2 in PCD (20.9±8.0, p<0.05) but not CF (88.1±37.0 nanomol/min).Conclusions: Children with PCD differ from CF patients in their responses to treatment of a mild pulmonary exacerbation. While lung function improved in both groups, neutrophil elastase persisted at higher levels in CF at the end of treatment. ER -