RT Journal Article
SR Electronic
T1 Changes in airway inflammation during pulmonary exacerbations in patients with cystic fibrosis and primary ciliary dyskinesia
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP ERJ-01390-2015
DO 10.1183/13993003.01390-2015
A1 Felix Ratjen
A1 Valerie Waters
A1 Michelle Klingel
A1 Nancy McDonald
A1 Sharon Dell
A1 Timothy Ronan Leahy
A1 Yvonne Yau
A1 Hartmut Grasemann
YR 2015
UL http://erj.ersjournals.com/content/early/2015/11/19/13993003.01390-2015.abstract
AB Lung disease in patients with both primary ciliary dyskinesia (PCD) or cystic fibrosis (CF) is associated with impaired mucociliary clearance; however, clinical outcomes are typically worse in CF patients. We assessed whether CF and PCD patients differ in inflammatory response in the airways during pulmonary exacerbation.We first studied clinically stable PCD patients with a spectrum of bacterial pathogens to assess inflammatory response to different pathogens. Subsequently, PCD and CF patients with similar bacterial pathogens were studied at the time of a pulmonary exacerbation and after 21 days of antibiotics treatment. Qualitative and quantitative microbiology, cell counts, interleukin-8 concentrations, and neutrophil elastase activity were assessed in sputum samples obtained before and after treatment.In stable PCD patients, no significant differences were found in sputum inflammatory markers between individuals colonised with different bacterial pathogens. Pulmonary exacerbation severity assessed by a pulmonary exacerbation score and lung function decline from their previous baseline did not differ between CF and PCD patients. Bacterial density for Staphylococcus aureus and Haemophilus influenzae was higher in CF versus PCD (p<0.05), but absolute neutrophil counts were higher in PCD patients (p=0.02). While sputum elastase activity was similar in PCD and CF at the time of exacerbation, it decreased with antibiotic therapy in PCD (p<0.05) but not CF patients.PCD patients differ from those with CF in their responses to treatment of pulmonary exacerbations, with higher neutrophil elastase activity persisting in the CF airways at the end of treatment.PCD patients differ from those with CF in their responses to treatment of pulmonary exacerbations http://ow.ly/TtM54