Clinical Outcomes of Chronic Airway Infection in Primary Ciliary Dyskinesia.

INTRODUCTION: Primary ciliary dyskinesia (PCD) is a rare disorder of impaired respiratory mucociliary clearance and chronic pulmonary infections. The clinical impact of chronic infection is poorly understood. The objective of this single-center, retrospective cohort study was to assess clinical outcomes of people with PCD and chronic airway infection. We hypothesized that chronic airway infection would be associated with greater declines in lung function and higher pulmonary exacerbation rates. METHODS: Linear mixed effects models were used to analyze the association of chronic infection with percent predicted forced expiratory volume in 1 s (ppFEV1). Poisson mixed models were used to analyze the association of chronic infection with exacerbation rates. RESULTS: Thirty-two people with PCD were included: 19 (59%) with chronic infection including 5 (16%) with chronic Pseudomonas aeruginosa (PA), 10 (31%) with chronic Haemophilus influenzae (HI), 2 (6%) with chronic methicillin-sensitive Staphylococcus aureus (MSSA) and 2 (6%) with chronic Aspergillus fumigatus (AF). A lower ppFEV1 was associated with chronic AF, but not with other chronic infections. The exacerbation rate ratio was 1.29 [95% CI 0.72, 2.30] (p = 0.388), 5.03 [95% CI 1.41, 18.0] (p = 0.013), 0.41 [95% CI 0.16, 1.08] (p = 0.072) and 2.19 [95% CI 0.39, 12.30] (p = 0.373) after development of chronic HI infection, chronic PA infection, chronic MSSA infection and chronic AF infection, respectively. CONCLUSION: In this single-center, retrospective study of chronic airway infections in PCD, chronic infection with PA was associated with a greater rate of pulmonary exacerbations but ppFEV1 decline was only associated with chronic AF infection.