Prevalence and course of disease after lung resection in primary ciliary dyskinesia: A cohort & nested case-control study

Panayiotis Kouis; Myrofora Goutaki; Florian S. Halbeisen; Ifigeneia Gioti; Nicos Middleton; Israel Amirav; Angelo Barbato; Laura Behan; Mieke Boon; Nagehan Emiralioglu; Eric G. Haarman; Bulent Karadag; Cordula Koerner-Rettberg; Romain Lazor; Michael R. Loebinger; Bernard Maitre; Henryk Mazurek; Lucy Morgan; Kim Gjerum Nielsen; Heymut Omran; Ugur Özçelik; Mareike Price; Andrzej Pogorzelski; Deborah Snijders; Guillaume Thouvenin; Claudius Werner; Zorica Zivkovic; Claudia E. Kuehni; Panayiotis K. Yiallouros

doi:10.1186/s12931-019-1183-y

Prevalence and course of disease after lung resection in primary ciliary dyskinesia: A cohort & nested case-control study

Panayiotis Kouis, Myrofora Goutaki, Florian S. Halbeisen, Ifigeneia Gioti, Nicos Middleton, Israel Amirav, Angelo Barbato, Laura Behan, Mieke Boon, Nagehan Emiralioglu, Eric G. Haarman, Bulent Karadag, Cordula Koerner-Rettberg, Romain Lazor, Michael R. Loebinger, Bernard Maitre, Henryk Mazurek, Lucy Morgan, Kim Gjerum Nielsen, Heymut OmranUgur Özçelik, Mareike Price, Andrzej Pogorzelski, Deborah Snijders, Guillaume Thouvenin, Claudius Werner, Zorica Zivkovic, Claudia E. Kuehni, Panayiotis K. Yiallouros

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Abstract

Background: Lung resection is a controversial and understudied therapeutic modality in Primary Ciliary Dyskinesia (PCD). We assessed the prevalence of lung resection in PCD across countries and compared disease course in lobectomised and non-lobectomised patients. Methods: In the international iPCD cohort, we identified lobectomised and non-lobectomised age and sex-matched PCD patients and compared their characteristics, lung function and BMI cross-sectionally and longitudinally. Results: Among 2896 patients in the iPCD cohort, 163 from 20 centers (15 countries) underwent lung resection (5.6%). Among adult patients, prevalence of lung resection was 8.9%, demonstrating wide variation among countries. Compared to the rest of the iPCD cohort, lobectomised patients were more often females, older at diagnosis, and more often had situs solitus. In about half of the cases (45.6%) lung resection was performed before presentation to specialized PCD centers for diagnostic work-up. Compared to controls (n = 197), lobectomised patients had lower FVC z-scores (-2.41 vs-1.35, p = 0.0001) and FEV1 z-scores (-2.79 vs-1.99, p = 0.003) at their first post-lung resection assessment. After surgery, lung function continued to decline at a faster rate in lobectomised patients compared to controls (FVC z-score slope:-0.037/year Vs-0.009/year, p = 0.047 and FEV1 z-score slope:-0.052/year Vs-0.033/year, p = 0.235), although difference did not reach statistical significance for FEV1. Within cases, females and patients with multiple lobe resections had lower lung function. Conclusions: Prevalence of lung resection in PCD varies widely between countries, is often performed before PCD diagnosis and overall is more frequent in patients with delayed diagnosis. After lung resection, compared to controls most lobectomised patients have poorer and continuing decline of lung function despite lung resection. Further studies benefiting from prospective data collection are needed to confirm these findings.

Original language	English
Article number	212
Journal	Respiratory Research
Volume	20
Issue number	1
DOIs	https://doi.org/10.1186/s12931-019-1183-y
State	Published - 18 Sep 2019
Externally published	Yes

Bibliographical note

Publisher Copyright:
© 2019 The Author(s).

Funding

This study was supported by EU 7th Framework Program EC-GA No. 305404 BESTCILIA and COST action BEAT-PCD (BM1407). The primary ciliary dyskinesia (PCD) research at the Institute of Social and Preventive Medicine (ISPM) in Bern is supported by the Swiss National Science Foundation (SNF 320030_173044). This work was supported by Ministry of Science Republic of Serbia, grant No III 41004. In France, this work was supported by the Institut National de la Sante et de la Racherche Medicale (INSERM), the RaDiCO funded by the French National Research Agency under the specific program "Investments for the future" (Cohort grant agreement ANR-10-COHO-0003). The sponsors had no role in the design of the study, the collection and analysis of the data, or the preparation of the manuscript.

Funders	Funder number
EU 7th Framework Program
Seventh Framework Programme	305404
European Cooperation in Science and Technology	BM1407
Agence Nationale de la Recherche	ANR-10-COHO-0003
Institut national de la santé et de la recherche médicale
Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung	320030_173044
Ministarstvo Prosvete, Nauke i Tehnološkog Razvoja	III 41004

Keywords

Ciliary motility disorders (MeSH)
Kartagener syndrome (MeSH)
Lobectomy

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10.1186/s12931-019-1183-y

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Kouis, P., Goutaki, M., Halbeisen, F. S., Gioti, I., Middleton, N., Amirav, I., Barbato, A., Behan, L., Boon, M., Emiralioglu, N., Haarman, E. G., Karadag, B., Koerner-Rettberg, C., Lazor, R., Loebinger, M. R., Maitre, B., Mazurek, H., Morgan, L., Nielsen, K. G., ... Yiallouros, P. K. (2019). Prevalence and course of disease after lung resection in primary ciliary dyskinesia: A cohort & nested case-control study. Respiratory Research, 20(1), Article 212. https://doi.org/10.1186/s12931-019-1183-y

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title = "Prevalence and course of disease after lung resection in primary ciliary dyskinesia: A cohort & nested case-control study",

abstract = "Background: Lung resection is a controversial and understudied therapeutic modality in Primary Ciliary Dyskinesia (PCD). We assessed the prevalence of lung resection in PCD across countries and compared disease course in lobectomised and non-lobectomised patients. Methods: In the international iPCD cohort, we identified lobectomised and non-lobectomised age and sex-matched PCD patients and compared their characteristics, lung function and BMI cross-sectionally and longitudinally. Results: Among 2896 patients in the iPCD cohort, 163 from 20 centers (15 countries) underwent lung resection (5.6%). Among adult patients, prevalence of lung resection was 8.9%, demonstrating wide variation among countries. Compared to the rest of the iPCD cohort, lobectomised patients were more often females, older at diagnosis, and more often had situs solitus. In about half of the cases (45.6%) lung resection was performed before presentation to specialized PCD centers for diagnostic work-up. Compared to controls (n = 197), lobectomised patients had lower FVC z-scores (-2.41 vs-1.35, p = 0.0001) and FEV1 z-scores (-2.79 vs-1.99, p = 0.003) at their first post-lung resection assessment. After surgery, lung function continued to decline at a faster rate in lobectomised patients compared to controls (FVC z-score slope:-0.037/year Vs-0.009/year, p = 0.047 and FEV1 z-score slope:-0.052/year Vs-0.033/year, p = 0.235), although difference did not reach statistical significance for FEV1. Within cases, females and patients with multiple lobe resections had lower lung function. Conclusions: Prevalence of lung resection in PCD varies widely between countries, is often performed before PCD diagnosis and overall is more frequent in patients with delayed diagnosis. After lung resection, compared to controls most lobectomised patients have poorer and continuing decline of lung function despite lung resection. Further studies benefiting from prospective data collection are needed to confirm these findings.",

keywords = "Ciliary motility disorders (MeSH), Kartagener syndrome (MeSH), Lobectomy",

author = "Panayiotis Kouis and Myrofora Goutaki and Halbeisen, {Florian S.} and Ifigeneia Gioti and Nicos Middleton and Israel Amirav and Angelo Barbato and Laura Behan and Mieke Boon and Nagehan Emiralioglu and Haarman, {Eric G.} and Bulent Karadag and Cordula Koerner-Rettberg and Romain Lazor and Loebinger, {Michael R.} and Bernard Maitre and Henryk Mazurek and Lucy Morgan and Nielsen, {Kim Gjerum} and Heymut Omran and Ugur {\"O}z{\c c}elik and Mareike Price and Andrzej Pogorzelski and Deborah Snijders and Guillaume Thouvenin and Claudius Werner and Zorica Zivkovic and Kuehni, {Claudia E.} and Yiallouros, {Panayiotis K.}",

note = "Publisher Copyright: {\textcopyright} 2019 The Author(s).",

year = "2019",

month = sep,

day = "18",

doi = "10.1186/s12931-019-1183-y",

language = "אנגלית",

volume = "20",

journal = "Respiratory Research",

issn = "1465-9921",

publisher = "BioMed Central Ltd.",

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Kouis, P, Goutaki, M, Halbeisen, FS, Gioti, I, Middleton, N, Amirav, I, Barbato, A, Behan, L, Boon, M, Emiralioglu, N, Haarman, EG, Karadag, B, Koerner-Rettberg, C, Lazor, R, Loebinger, MR, Maitre, B, Mazurek, H, Morgan, L, Nielsen, KG, Omran, H, Özçelik, U, Price, M, Pogorzelski, A, Snijders, D, Thouvenin, G, Werner, C, Zivkovic, Z, Kuehni, CE & Yiallouros, PK 2019, 'Prevalence and course of disease after lung resection in primary ciliary dyskinesia: A cohort & nested case-control study', Respiratory Research, vol. 20, no. 1, 212. https://doi.org/10.1186/s12931-019-1183-y

TY - JOUR

T1 - Prevalence and course of disease after lung resection in primary ciliary dyskinesia

T2 - A cohort & nested case-control study

AU - Kouis, Panayiotis

AU - Goutaki, Myrofora

AU - Halbeisen, Florian S.

AU - Gioti, Ifigeneia

AU - Middleton, Nicos

AU - Amirav, Israel

AU - Barbato, Angelo

AU - Behan, Laura

AU - Boon, Mieke

AU - Emiralioglu, Nagehan

AU - Haarman, Eric G.

AU - Karadag, Bulent

AU - Koerner-Rettberg, Cordula

AU - Lazor, Romain

AU - Loebinger, Michael R.

AU - Maitre, Bernard

AU - Mazurek, Henryk

AU - Morgan, Lucy

AU - Nielsen, Kim Gjerum

AU - Omran, Heymut

AU - Özçelik, Ugur

AU - Price, Mareike

AU - Pogorzelski, Andrzej

AU - Snijders, Deborah

AU - Thouvenin, Guillaume

AU - Werner, Claudius

AU - Zivkovic, Zorica

AU - Kuehni, Claudia E.

AU - Yiallouros, Panayiotis K.

PY - 2019/9/18

Y1 - 2019/9/18

N2 - Background: Lung resection is a controversial and understudied therapeutic modality in Primary Ciliary Dyskinesia (PCD). We assessed the prevalence of lung resection in PCD across countries and compared disease course in lobectomised and non-lobectomised patients. Methods: In the international iPCD cohort, we identified lobectomised and non-lobectomised age and sex-matched PCD patients and compared their characteristics, lung function and BMI cross-sectionally and longitudinally. Results: Among 2896 patients in the iPCD cohort, 163 from 20 centers (15 countries) underwent lung resection (5.6%). Among adult patients, prevalence of lung resection was 8.9%, demonstrating wide variation among countries. Compared to the rest of the iPCD cohort, lobectomised patients were more often females, older at diagnosis, and more often had situs solitus. In about half of the cases (45.6%) lung resection was performed before presentation to specialized PCD centers for diagnostic work-up. Compared to controls (n = 197), lobectomised patients had lower FVC z-scores (-2.41 vs-1.35, p = 0.0001) and FEV1 z-scores (-2.79 vs-1.99, p = 0.003) at their first post-lung resection assessment. After surgery, lung function continued to decline at a faster rate in lobectomised patients compared to controls (FVC z-score slope:-0.037/year Vs-0.009/year, p = 0.047 and FEV1 z-score slope:-0.052/year Vs-0.033/year, p = 0.235), although difference did not reach statistical significance for FEV1. Within cases, females and patients with multiple lobe resections had lower lung function. Conclusions: Prevalence of lung resection in PCD varies widely between countries, is often performed before PCD diagnosis and overall is more frequent in patients with delayed diagnosis. After lung resection, compared to controls most lobectomised patients have poorer and continuing decline of lung function despite lung resection. Further studies benefiting from prospective data collection are needed to confirm these findings.

AB - Background: Lung resection is a controversial and understudied therapeutic modality in Primary Ciliary Dyskinesia (PCD). We assessed the prevalence of lung resection in PCD across countries and compared disease course in lobectomised and non-lobectomised patients. Methods: In the international iPCD cohort, we identified lobectomised and non-lobectomised age and sex-matched PCD patients and compared their characteristics, lung function and BMI cross-sectionally and longitudinally. Results: Among 2896 patients in the iPCD cohort, 163 from 20 centers (15 countries) underwent lung resection (5.6%). Among adult patients, prevalence of lung resection was 8.9%, demonstrating wide variation among countries. Compared to the rest of the iPCD cohort, lobectomised patients were more often females, older at diagnosis, and more often had situs solitus. In about half of the cases (45.6%) lung resection was performed before presentation to specialized PCD centers for diagnostic work-up. Compared to controls (n = 197), lobectomised patients had lower FVC z-scores (-2.41 vs-1.35, p = 0.0001) and FEV1 z-scores (-2.79 vs-1.99, p = 0.003) at their first post-lung resection assessment. After surgery, lung function continued to decline at a faster rate in lobectomised patients compared to controls (FVC z-score slope:-0.037/year Vs-0.009/year, p = 0.047 and FEV1 z-score slope:-0.052/year Vs-0.033/year, p = 0.235), although difference did not reach statistical significance for FEV1. Within cases, females and patients with multiple lobe resections had lower lung function. Conclusions: Prevalence of lung resection in PCD varies widely between countries, is often performed before PCD diagnosis and overall is more frequent in patients with delayed diagnosis. After lung resection, compared to controls most lobectomised patients have poorer and continuing decline of lung function despite lung resection. Further studies benefiting from prospective data collection are needed to confirm these findings.

KW - Ciliary motility disorders (MeSH)

KW - Kartagener syndrome (MeSH)

KW - Lobectomy

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SN - 1465-9921

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JO - Respiratory Research

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Prevalence and course of disease after lung resection in primary ciliary dyskinesia: A cohort & nested case-control study

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Keywords

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