Risk factors for the deterioration of pulmonary function in primary ciliary dyskinesia

Abstract

Background: PCD is a genetic disease leading to a decline in pulmonary function. There is only little knowledge of factors determining the long-term pulmonary outcome. Especially adherence has not been addressed yet although being an independent risk factor for an increased loss of lung capacity in other chronic respiratory diseases. Objective: Assessing the impact of bacterial airway colonization and adherence on long-term lung function in patients with PCD. Methods: Data on colonization and lung function parameters like forced expiratory volume in the first second (FEV1, Z-score) and lung clearance index (LCI2,5%) were collected for 7.01 ± 2.2 years (893 quarters) in 44 PCD patients. Adherence was classified as good, moderate or poor. The impact of both adherence and colonization was assessed for the long-term course of FEV1, the association of colonization with lung function also quarterly. Statistics: Kruskall–Wallis test, T test, ANOVA, linear regression, linear mixed model. Results: Chronic colonization did not show any impact on the for long-term course of FEV1, but adherence was a significant factor: patients with good adherence showed better FEV1 at the end of the observation period than children with poor adherence (–0.15 ± 0.88 vs. –2.63 ± 1.79, p < 0.01). Conclusion: Adherence has not yet been investigated for PCD. However, we found it to be a major significant factor affecting long-term FEV1 in PCD. Thus, it should be taken into consideration in the treatment protocols for PCD.