Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study

Siobhán B. Carr; Elliot McClenaghan; Alexander Elbert; Albert Faro; Rebecca Cosgriff; Olzhas Abdrakhmanov; Keith Brownlee; Pierre Régis Burgel; Catherine A. Byrnes; Stephanie Y. Cheng; Carla Colombo; Harriet Corvol; Géraldine Daneau; Christopher H. Goss; Vincent Gulmans; Hector Gutierrez; Satenik Harutyunyan; Meagan Helmick; Andreas Jung; Nataliya Kashirskaya; Edward McKone; Joel Melo; Peter G. Middleton; Pedro Mondejar-Lopez; Isabelle de Monestrol; Lutz Nährlich; Rita Padoan; Megan Parker; M. Dolores Pastor-Vivero; Samar Rizvi; Rasa Ruseckaite; Marco Salvatore; Luiz Vicente R.F. da Silva-Filho; Nick Versmessen; Marco Zampoli; Bruce C. Marshall; Anne L. Stephenson; Scott C. Bell; David Reid; Peter Wark; Eva Van Braeckel; Sophie Gohy; Christiane Knoop; Jessica Pirson; Elke De Wachter; Lieven Dupont; Laurence Hanssens; Vicky Nowé; Monique Lequesne; Rodrigo A. Athanazio; CF Registry Global Collaboration; Owen Dempsey

doi:10.1016/j.jcf.2022.06.006

Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study

Siobhán B. Carr^* (Corresponding Author), Elliot McClenaghan, Alexander Elbert, Albert Faro, Rebecca Cosgriff, Olzhas Abdrakhmanov, Keith Brownlee, Pierre Régis Burgel, Catherine A. Byrnes, Stephanie Y. Cheng, Carla Colombo, Harriet Corvol, Géraldine Daneau, Christopher H. Goss, Vincent Gulmans, Hector Gutierrez, Satenik Harutyunyan, Meagan Helmick, Andreas Jung, Nataliya KashirskayaEdward McKone, Joel Melo, Peter G. Middleton, Pedro Mondejar-Lopez, Isabelle de Monestrol, Lutz Nährlich, Rita Padoan, Megan Parker, M. Dolores Pastor-Vivero, Samar Rizvi, Rasa Ruseckaite, Marco Salvatore, Luiz Vicente R.F. da Silva-Filho, Nick Versmessen, Marco Zampoli, Bruce C. Marshall, Anne L. Stephenson, Scott C. Bell, David Reid, Peter Wark, Eva Van Braeckel, Sophie Gohy, Christiane Knoop, Jessica Pirson, Elke De Wachter, Lieven Dupont, Laurence Hanssens, Vicky Nowé, Monique Lequesne, Rodrigo A. Athanazio, CF Registry Global Collaboration, Owen Dempsey

^*Corresponding author for this work

Medical Education

Research output: Contribution to journal › Article › peer-review

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Abstract

Background: This international study aimed to characterise the impact of acute SARS-CoV-2 infection in people with cystic fibrosis and investigate factors associated with severe outcomes. Methods Data from 22 countries prior to 13^th December 2020 and the introduction of vaccines were included. It was de-identified and included patient demographics, clinical characteristics, treatments, outcomes and sequalae following SARS-CoV-2 infection. Multivariable logistic regression was used to investigate factors associated with clinical progression to severe COVID-19, using the primary outcome of hospitalisation with supplemental oxygen. Results: SARS-CoV-2 was reported in 1555 people with CF, 1452 were included in the analysis. One third were aged <18 years, and 9.4% were solid-organ transplant recipients. 74.5% were symptomatic and 22% were admitted to hospital. In the non-transplanted cohort, 39.5% of patients with ppFEV1<40% were hospitalised with oxygen verses 3.2% with ppFEV >70%: a 17-fold increase in odds. Worse outcomes were independently associated with older age, non-white race, underweight body mass index, and CF-related diabetes. Prescription of highly effective CFTR modulator therapies was associated with a significantly reduced odds of being hospitalised with oxygen (AOR 0.43 95%CI 0.31-0.60 p<0.001). Transplanted patients were hospitalised with supplemental oxygen therapy (21.9%) more often than non-transplanted (8.8%) and was independently associated with the primary outcome (Adjusted OR 2.45 95%CI 1.27-4.71 p=0.007). Conclusions: This is the first study to show that there is a protective effect from the use of CFTR modulator therapy and that people with CF from an ethnic minority are at more risk of severe infection with SARS-CoV-2.

Original language	English
Pages (from-to)	e221-e231
Number of pages	11
Journal	Journal of Cystic Fibrosis
Volume	21
Issue number	4
Early online date	22 Jul 2022
DOIs	https://doi.org/10.1016/j.jcf.2022.06.006
Publication status	Published - 22 Jul 2022

Bibliographical note

Funding Information:
AS, RC, have funding from Canadian Institutes of Health Research to support the Global Registry Collaboration on Covid and CF.

Acknowledgements:
We would like to thank the people with cystic fibrosis around the world that have consented to have their anonymised data collected by their respective CF Registries. The local and National CF teams that have worked hard to collect and clean the data.

Data Availability Statement

Supplementary Materials:
Supplementary material associated with this article can be found, in the online version, at doi:10.1016/j.jcf.2022.06.006.

Keywords

Coronavirus
COVID-19
Cystic fibrosis
SARS-CoV-2
Transplant

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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Carr, S. B., McClenaghan, E., Elbert, A., Faro, A., Cosgriff, R., Abdrakhmanov, O., Brownlee, K., Burgel, P. R., Byrnes, C. A., Cheng, S. Y., Colombo, C., Corvol, H., Daneau, G., Goss, C. H., Gulmans, V., Gutierrez, H., Harutyunyan, S., Helmick, M., Jung, A., ... Dempsey, O. (2022). Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study. Journal of Cystic Fibrosis, 21(4), e221-e231. https://doi.org/10.1016/j.jcf.2022.06.006

Carr, SB, McClenaghan, E, Elbert, A, Faro, A, Cosgriff, R, Abdrakhmanov, O, Brownlee, K, Burgel, PR, Byrnes, CA, Cheng, SY, Colombo, C, Corvol, H, Daneau, G, Goss, CH, Gulmans, V, Gutierrez, H, Harutyunyan, S, Helmick, M, Jung, A, Kashirskaya, N, McKone, E, Melo, J, Middleton, PG, Mondejar-Lopez, P, de Monestrol, I, Nährlich, L, Padoan, R, Parker, M, Pastor-Vivero, MD, Rizvi, S, Ruseckaite, R, Salvatore, M, da Silva-Filho, LVRF, Versmessen, N, Zampoli, M, Marshall, BC, Stephenson, AL, Bell, SC, Reid, D, Wark, P, Van Braeckel, E, Gohy, S, Knoop, C, Pirson, J, De Wachter, E, Dupont, L, Hanssens, L, Nowé, V, Lequesne, M, Athanazio, RA, CF Registry Global Collaboration & Dempsey, O 2022, 'Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study', Journal of Cystic Fibrosis, vol. 21, no. 4, pp. e221-e231. https://doi.org/10.1016/j.jcf.2022.06.006

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abstract = "Background: This international study aimed to characterise the impact of acute SARS-CoV-2 infection in people with cystic fibrosis and investigate factors associated with severe outcomes. Methods Data from 22 countries prior to 13th December 2020 and the introduction of vaccines were included. It was de-identified and included patient demographics, clinical characteristics, treatments, outcomes and sequalae following SARS-CoV-2 infection. Multivariable logistic regression was used to investigate factors associated with clinical progression to severe COVID-19, using the primary outcome of hospitalisation with supplemental oxygen. Results: SARS-CoV-2 was reported in 1555 people with CF, 1452 were included in the analysis. One third were aged <18 years, and 9.4% were solid-organ transplant recipients. 74.5% were symptomatic and 22% were admitted to hospital. In the non-transplanted cohort, 39.5% of patients with ppFEV1<40% were hospitalised with oxygen verses 3.2% with ppFEV >70%: a 17-fold increase in odds. Worse outcomes were independently associated with older age, non-white race, underweight body mass index, and CF-related diabetes. Prescription of highly effective CFTR modulator therapies was associated with a significantly reduced odds of being hospitalised with oxygen (AOR 0.43 95%CI 0.31-0.60 p<0.001). Transplanted patients were hospitalised with supplemental oxygen therapy (21.9%) more often than non-transplanted (8.8%) and was independently associated with the primary outcome (Adjusted OR 2.45 95%CI 1.27-4.71 p=0.007). Conclusions: This is the first study to show that there is a protective effect from the use of CFTR modulator therapy and that people with CF from an ethnic minority are at more risk of severe infection with SARS-CoV-2.",

keywords = "Coronavirus, COVID-19, Cystic fibrosis, SARS-CoV-2, Transplant",

author = "Carr, {Siobh{\'a}n B.} and Elliot McClenaghan and Alexander Elbert and Albert Faro and Rebecca Cosgriff and Olzhas Abdrakhmanov and Keith Brownlee and Burgel, {Pierre R{\'e}gis} and Byrnes, {Catherine A.} and Cheng, {Stephanie Y.} and Carla Colombo and Harriet Corvol and G{\'e}raldine Daneau and Goss, {Christopher H.} and Vincent Gulmans and Hector Gutierrez and Satenik Harutyunyan and Meagan Helmick and Andreas Jung and Nataliya Kashirskaya and Edward McKone and Joel Melo and Middleton, {Peter G.} and Pedro Mondejar-Lopez and {de Monestrol}, Isabelle and Lutz N{\"a}hrlich and Rita Padoan and Megan Parker and Pastor-Vivero, {M. Dolores} and Samar Rizvi and Rasa Ruseckaite and Marco Salvatore and {da Silva-Filho}, {Luiz Vicente R.F.} and Nick Versmessen and Marco Zampoli and Marshall, {Bruce C.} and Stephenson, {Anne L.} and Bell, {Scott C.} and David Reid and Peter Wark and {Van Braeckel}, Eva and Sophie Gohy and Christiane Knoop and Jessica Pirson and {De Wachter}, Elke and Lieven Dupont and Laurence Hanssens and Vicky Now{\'e} and Monique Lequesne and Athanazio, {Rodrigo A.} and {CF Registry Global Collaboration} and Owen Dempsey",

note = "Funding Information: AS, RC, have funding from Canadian Institutes of Health Research to support the Global Registry Collaboration on Covid and CF. Acknowledgements: We would like to thank the people with cystic fibrosis around the world that have consented to have their anonymised data collected by their respective CF Registries. The local and National CF teams that have worked hard to collect and clean the data.",

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day = "22",

doi = "10.1016/j.jcf.2022.06.006",

language = "English",

volume = "21",

pages = "e221--e231",

journal = "Journal of Cystic Fibrosis",

issn = "1569-1993",

publisher = "Elsevier",

number = "4",

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TY - JOUR

T1 - Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis

T2 - A global observational study

AU - Carr, Siobhán B.

AU - McClenaghan, Elliot

AU - Elbert, Alexander

AU - Faro, Albert

AU - Cosgriff, Rebecca

AU - Abdrakhmanov, Olzhas

AU - Brownlee, Keith

AU - Burgel, Pierre Régis

AU - Byrnes, Catherine A.

AU - Cheng, Stephanie Y.

AU - Colombo, Carla

AU - Corvol, Harriet

AU - Daneau, Géraldine

AU - Goss, Christopher H.

AU - Gulmans, Vincent

AU - Gutierrez, Hector

AU - Harutyunyan, Satenik

AU - Helmick, Meagan

AU - Jung, Andreas

AU - Kashirskaya, Nataliya

AU - McKone, Edward

AU - Melo, Joel

AU - Middleton, Peter G.

AU - Mondejar-Lopez, Pedro

AU - de Monestrol, Isabelle

AU - Nährlich, Lutz

AU - Padoan, Rita

AU - Parker, Megan

AU - Pastor-Vivero, M. Dolores

AU - Rizvi, Samar

AU - Ruseckaite, Rasa

AU - Salvatore, Marco

AU - da Silva-Filho, Luiz Vicente R.F.

AU - Versmessen, Nick

AU - Zampoli, Marco

AU - Marshall, Bruce C.

AU - Stephenson, Anne L.

AU - Bell, Scott C.

AU - Reid, David

AU - Wark, Peter

AU - Van Braeckel, Eva

AU - Gohy, Sophie

AU - Knoop, Christiane

AU - Pirson, Jessica

AU - De Wachter, Elke

AU - Dupont, Lieven

AU - Hanssens, Laurence

AU - Nowé, Vicky

AU - Lequesne, Monique

AU - Athanazio, Rodrigo A.

AU - CF Registry Global Collaboration

AU - Dempsey, Owen

N1 - Funding Information: AS, RC, have funding from Canadian Institutes of Health Research to support the Global Registry Collaboration on Covid and CF. Acknowledgements: We would like to thank the people with cystic fibrosis around the world that have consented to have their anonymised data collected by their respective CF Registries. The local and National CF teams that have worked hard to collect and clean the data.

PY - 2022/7/22

Y1 - 2022/7/22

N2 - Background: This international study aimed to characterise the impact of acute SARS-CoV-2 infection in people with cystic fibrosis and investigate factors associated with severe outcomes. Methods Data from 22 countries prior to 13th December 2020 and the introduction of vaccines were included. It was de-identified and included patient demographics, clinical characteristics, treatments, outcomes and sequalae following SARS-CoV-2 infection. Multivariable logistic regression was used to investigate factors associated with clinical progression to severe COVID-19, using the primary outcome of hospitalisation with supplemental oxygen. Results: SARS-CoV-2 was reported in 1555 people with CF, 1452 were included in the analysis. One third were aged <18 years, and 9.4% were solid-organ transplant recipients. 74.5% were symptomatic and 22% were admitted to hospital. In the non-transplanted cohort, 39.5% of patients with ppFEV1<40% were hospitalised with oxygen verses 3.2% with ppFEV >70%: a 17-fold increase in odds. Worse outcomes were independently associated with older age, non-white race, underweight body mass index, and CF-related diabetes. Prescription of highly effective CFTR modulator therapies was associated with a significantly reduced odds of being hospitalised with oxygen (AOR 0.43 95%CI 0.31-0.60 p<0.001). Transplanted patients were hospitalised with supplemental oxygen therapy (21.9%) more often than non-transplanted (8.8%) and was independently associated with the primary outcome (Adjusted OR 2.45 95%CI 1.27-4.71 p=0.007). Conclusions: This is the first study to show that there is a protective effect from the use of CFTR modulator therapy and that people with CF from an ethnic minority are at more risk of severe infection with SARS-CoV-2.

AB - Background: This international study aimed to characterise the impact of acute SARS-CoV-2 infection in people with cystic fibrosis and investigate factors associated with severe outcomes. Methods Data from 22 countries prior to 13th December 2020 and the introduction of vaccines were included. It was de-identified and included patient demographics, clinical characteristics, treatments, outcomes and sequalae following SARS-CoV-2 infection. Multivariable logistic regression was used to investigate factors associated with clinical progression to severe COVID-19, using the primary outcome of hospitalisation with supplemental oxygen. Results: SARS-CoV-2 was reported in 1555 people with CF, 1452 were included in the analysis. One third were aged <18 years, and 9.4% were solid-organ transplant recipients. 74.5% were symptomatic and 22% were admitted to hospital. In the non-transplanted cohort, 39.5% of patients with ppFEV1<40% were hospitalised with oxygen verses 3.2% with ppFEV >70%: a 17-fold increase in odds. Worse outcomes were independently associated with older age, non-white race, underweight body mass index, and CF-related diabetes. Prescription of highly effective CFTR modulator therapies was associated with a significantly reduced odds of being hospitalised with oxygen (AOR 0.43 95%CI 0.31-0.60 p<0.001). Transplanted patients were hospitalised with supplemental oxygen therapy (21.9%) more often than non-transplanted (8.8%) and was independently associated with the primary outcome (Adjusted OR 2.45 95%CI 1.27-4.71 p=0.007). Conclusions: This is the first study to show that there is a protective effect from the use of CFTR modulator therapy and that people with CF from an ethnic minority are at more risk of severe infection with SARS-CoV-2.

KW - Coronavirus

KW - COVID-19

KW - Cystic fibrosis

KW - SARS-CoV-2

KW - Transplant

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UR - https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9189103/

U2 - 10.1016/j.jcf.2022.06.006

DO - 10.1016/j.jcf.2022.06.006

M3 - Article

C2 - 35753987

AN - SCOPUS:85135282334

SN - 1569-1993

VL - 21

SP - e221-e231

JO - Journal of Cystic Fibrosis

JF - Journal of Cystic Fibrosis

IS - 4

ER -

Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study

Abstract

Bibliographical note

Data Availability Statement

Keywords

UN SDGs

Access to Document

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