Lung function in patients with Primary Ciliary Dyskinesia: an iPCD Cohort study.

Halbeisen, Florian S; Goutaki, Myrofora; Spycher, Ben D; Amirav, Israel; Behan, Laura; Boon, Mieke; Hogg, Claire; Casaulta, Carmen; Crowley, Suzanne; Haarman, Eric G; Karadag, Bulent; Koerner-Rettberg, Cordula; Loebinger, Michael R; Mazurek, Henryk; Morgan, Lucy; Nielsen, Kim G; Omran, Heymut; Santamaria, Francesca; Schwerk, Nicolaus; Thouvenin, Guillaume; ... (2018). Lung function in patients with Primary Ciliary Dyskinesia: an iPCD Cohort study. European respiratory journal, 52(2), p. 1801040. European Respiratory Society 10.1183/13993003.01040-2018

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Primary ciliary dyskinesia (PCD) has been considered to be relatively mild disease, especially compared to cystic fibrosis (CF), but studies on lung function in PCD patients have been few and small.This study compared lung function from spirometry of PCD patients to normal reference values and to published data from CF. We calculated z-scores and percentage of predicted values for FEV and FVC using the Global Lung Function Initiative 2012 for 991 patients from the international PCD (iPCD) Cohort. We then assessed associations with age, sex, country, diagnostic certainty, organ laterality, body mass index and age at diagnosis in linear regression models. Lung function in PCD patients was reduced compared to reference values in both sexes and all age groups. Children aged 6-9 years had the smallest impairment (FEV z-score -0.84 [-1.03 to -0.65], FVC z-score -0.31 [-0.51 to -0.11]). Compared to CF patients, FEV was similarly reduced in children (age 6-9 years PCD, 91% [88-93%]; CF, 90% [88-91%]), but less impaired in young adults (age 18-21 years PCD, 79% [76-82%]; CF, 66% [65-68%]). The results suggest that PCD affects lung function from early in life, which emphasizes the importance of early, standardised care for all patients.

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