Lung function in patients with primary ciliary dyskinesia: An iPCD Cohort study

Florian S. Halbeisen; Myrofora Goutaki; Ben D. Spycher; Israel Amirav; Laura Behan; Mieke Boon; Claire Hogg; Carmen Casaulta; Suzanne Crowley; Eric G. Haarman; Bulent Karadag; Cordula Koerner-Rettberg; Michael R. Loebinger; Henryk Mazurek; Lucy Morgan; Kim G. Nielsen; Heymut Omran; Francesca Santamaria; Nicolaus Schwerk; Guillaume Thouvenin; Panayiotis Yiallouros; Jane S. Lucas; Philipp Latzin; Claudia E. Kuehni

doi:10.1183/13993003.01040-2018

Lung function in patients with primary ciliary dyskinesia: An iPCD Cohort study

Florian S. Halbeisen, Myrofora Goutaki, Ben D. Spycher, Israel Amirav, Laura Behan, Mieke Boon, Claire Hogg, Carmen Casaulta, Suzanne Crowley, Eric G. Haarman, Bulent Karadag, Cordula Koerner-Rettberg, Michael R. Loebinger, Henryk Mazurek, Lucy Morgan, Kim G. Nielsen, Heymut Omran, Francesca Santamaria, Nicolaus Schwerk, Guillaume ThouveninPanayiotis Yiallouros, Jane S. Lucas, Philipp Latzin, Claudia E. Kuehni

Bar-Ilan University - Azrieli Faculty of Medicine

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66 Scopus citations

Abstract

Primary ciliary dyskinesia (PCD) has been considered a 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 patients. We calculated z-scores and % predicted values for forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 values for 991 patients from the international PCD 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 emphasises the importance of early standardised care for all patients.

Original language	English
Article number	1801040
Journal	European Respiratory Journal
Volume	52
Issue number	2
DOIs	https://doi.org/10.1183/13993003.01040-2018
State	Published - Aug 2018

Bibliographical note

Publisher Copyright:
Copyright ©ERS 2018.

Funding

Conflict of interest: N. Schwerk reports personal fees for lecturing from Novartis, Allergopharma and Infectopharm, and grants from FP7-ChILD EU, outside the submitted work. P. Yiallouros reports grants from European Union’s Seventh Framework Programme under EG-GA (number 35404 BESTCILIA), during the conduct of the study. P. Latzin reports personal fees from Gilead, Novartis, Polyphor, Roche, Santhera, Schwabe, Vertex, Vifor and Zambon, outside the submitted work. H. Mazurek reports grants from Bestcilia, during the conduct of the study. Support statement: This study is supported by Swiss National Science Foundation (320030_173044). The development of the iPCD Cohort has been funded from the European Union’s Seventh Framework Programme under EG-GA No.35404 BESTCILIA: Better Experimental Screening and Treatment for Primary Ciliary Dyskinesia. PCD research at ISPM Bern also receives national funding from the Lung Leagues of Bern, St. Gallen, Vaud, Ticino, and Valais, and the Milena-Carvajal Pro Kartagener Foundation. The researchers participate in the network of COST Action BEAT-PCD: Better Evidence to Advance Therapeutic options for PCD (BM 1407). B.D. Spycher was supported by a Swiss National Science Foundation fellowship (PZ00P3_147987). The National PCD Centre in Southampton is commissioned and funded by NHS England. Research in Southampton is supported by NIHR Southampton Biomedical Research Centre, NIHR Wellcome Trust Clinical Research Facility, National Institute for Health Research (RfPB PB-PG-1215–20014) and The AAIR Charity (Reg. No. 1129698). Funding information for this article has been deposited with the Crossref Funder Registry.

Funders	Funder number
Milena-Carvajal Pro Kartagener Foundation
Wellcome Trust
Heart of England NHS Foundation Trust
NIHR Bristol Biomedical Research Centre
National Institute for Health Research	1129698, RfPB PB-PG-1215–20014
Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung	35404, PZ00P3_147987, 320030_173044
Seventh Framework Programme	35404 BESTCILIA

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Halbeisen, F. S., Goutaki, M., Spycher, B. D., Amirav, I., Behan, L., Boon, M., Hogg, C., Casaulta, C., Crowley, S., Haarman, E. G., Karadag, B., Koerner-Rettberg, C., Loebinger, M. R., Mazurek, H., Morgan, L., Nielsen, K. G., Omran, H., Santamaria, F., Schwerk, N., ... Kuehni, C. E. (2018). Lung function in patients with primary ciliary dyskinesia: An iPCD Cohort study. European Respiratory Journal, 52(2), Article 1801040. https://doi.org/10.1183/13993003.01040-2018

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title = "Lung function in patients with primary ciliary dyskinesia: An iPCD Cohort study",

abstract = "Primary ciliary dyskinesia (PCD) has been considered a 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 patients. We calculated z-scores and % predicted values for forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 values for 991 patients from the international PCD 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 emphasises the importance of early standardised care for all patients.",

author = "Halbeisen, {Florian S.} and Myrofora Goutaki and Spycher, {Ben D.} and Israel Amirav and Laura Behan and Mieke Boon and Claire Hogg and Carmen Casaulta and Suzanne Crowley and Haarman, {Eric G.} and Bulent Karadag and Cordula Koerner-Rettberg and Loebinger, {Michael R.} and Henryk Mazurek and Lucy Morgan and Nielsen, {Kim G.} and Heymut Omran and Francesca Santamaria and Nicolaus Schwerk and Guillaume Thouvenin and Panayiotis Yiallouros and Lucas, {Jane S.} and Philipp Latzin and Kuehni, {Claudia E.}",

note = "Publisher Copyright: Copyright {\textcopyright}ERS 2018.",

year = "2018",

month = aug,

doi = "10.1183/13993003.01040-2018",

language = "אנגלית",

volume = "52",

journal = "European Respiratory Journal",

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Halbeisen, FS, Goutaki, M, Spycher, BD, Amirav, I, Behan, L, Boon, M, Hogg, C, Casaulta, C, Crowley, S, Haarman, EG, Karadag, B, Koerner-Rettberg, C, Loebinger, MR, Mazurek, H, Morgan, L, Nielsen, KG, Omran, H, Santamaria, F, Schwerk, N, Thouvenin, G, Yiallouros, P, Lucas, JS, Latzin, P & Kuehni, CE 2018, 'Lung function in patients with primary ciliary dyskinesia: An iPCD Cohort study', European Respiratory Journal, vol. 52, no. 2, 1801040. https://doi.org/10.1183/13993003.01040-2018

TY - JOUR

T1 - Lung function in patients with primary ciliary dyskinesia

T2 - An iPCD Cohort study

AU - Halbeisen, Florian S.

AU - Goutaki, Myrofora

AU - Spycher, Ben D.

AU - Amirav, Israel

AU - Behan, Laura

AU - Boon, Mieke

AU - Hogg, Claire

AU - Casaulta, Carmen

AU - Crowley, Suzanne

AU - Haarman, Eric G.

AU - Karadag, Bulent

AU - Koerner-Rettberg, Cordula

AU - Loebinger, Michael R.

AU - Mazurek, Henryk

AU - Morgan, Lucy

AU - Nielsen, Kim G.

AU - Omran, Heymut

AU - Santamaria, Francesca

AU - Schwerk, Nicolaus

AU - Thouvenin, Guillaume

AU - Yiallouros, Panayiotis

AU - Lucas, Jane S.

AU - Latzin, Philipp

AU - Kuehni, Claudia E.

PY - 2018/8

Y1 - 2018/8

N2 - Primary ciliary dyskinesia (PCD) has been considered a 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 patients. We calculated z-scores and % predicted values for forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 values for 991 patients from the international PCD 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 emphasises the importance of early standardised care for all patients.

AB - Primary ciliary dyskinesia (PCD) has been considered a 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 patients. We calculated z-scores and % predicted values for forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 values for 991 patients from the international PCD 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 emphasises the importance of early standardised care for all patients.

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Lung function in patients with primary ciliary dyskinesia: An iPCD Cohort study

Abstract

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