Abstract

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 FEV1 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 (FEV1 z-score −0.84 [−1.03 to −0.65], FVC z-score −0.31 [−0.51 to −0.11]). Compared to CF patients, FEV1 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.

Footnotes

Conflict of interest: Dr. Halbeisen has nothing to disclose.

Conflict of interest: Dr. Goutaki has nothing to disclose.

Conflict of interest: Dr. Spycher has nothing to disclose.

Conflict of interest: Dr. Amirav has nothing to disclose.

Conflict of interest: Dr. Behan has nothing to disclose.

Conflict of interest: Dr. Boon has nothing to disclose.

Conflict of interest: Dr. Hogg has nothing to disclose.

Conflict of interest: Dr. Casaulta has nothing to disclose.

Conflict of interest: Dr. Crowley has nothing to disclose.

Conflict of interest: Dr. Haarman has nothing to disclose.

Conflict of interest: Prof. Karadag has nothing to disclose.

Conflict of interest: Dr. Koerner-Rettberg has nothing to disclose.

Conflict of interest: Dr. Loebinger has nothing to disclose.

Conflict of interest: Dr. Mazurek reports grants from Bestcilia, during the conduct of the study;.

Conflict of interest: Dr. Morgan has nothing to disclose.

Conflict of interest: Dr. Nielsen has nothing to disclose.

Conflict of interest: Dr. Omran has nothing to disclose.

Conflict of interest: Dr. Santamaria has nothing to disclose.

Conflict of interest: Dr. Schwerk reports personal fees from paid lectures for the following companies: Novartis, Allergopharma, Infectopharm, grants from FP7-ChILD EU, outside the submitted work; .

Conflict of interest: Dr. Thouvenin has nothing to disclose.

Conflict of interest: Dr. Yiallouros reports grants from European Union's Seventh Framework Programme under EG-GA, during the conduct of the study; .

Conflict of interest: Dr. Lucas has nothing to disclose.

Conflict of interest: Dr. Latzin has nothing to disclose.

Conflict of interest: Prof. Kuehni has nothing to disclose.