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

Department of Clinical Medicine

18 Citations (Scopus)

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
ISSN	0903-1936
DOIs	https://doi.org/10.1183/13993003.01040-2018
Publication status	Published - 2018

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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

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, J, 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

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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 Jane s. Lucas and Philipp Latzin and Kuehni, {Claudia E.}",

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T1 - Lung function in patients with primary ciliary dyskinesia: 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

Y1 - 2018

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.

U2 - 10.1183/13993003.01040-2018

DO - 10.1183/13993003.01040-2018

M3 - Journal article

C2 - 30049738

SN - 0903-1936

VL - 52

JO - European Respiratory Journal

JF - European Respiratory Journal

IS - 2

M1 - 1801040

ER -

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

Abstract

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