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; CF Registry Global Collaboration; Geertjan Wesseling

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^*, 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, CF Registry Global Collaboration, Geertjan Wesseling

^*Corresponding author for this work

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

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.

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

Keywords

COVID-19/epidemiology
Cystic Fibrosis/complications
Cystic Fibrosis Transmembrane Conductance Regulator
Ethnicity
Humans
Minority Groups
Oxygen
SARS-CoV-2

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10.1016/j.jcf.2022.06.006

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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., ... Wesseling, G. (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

@article{0cb3fe09cd2a41798f296c9d25901cef,

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

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 = "COVID-19/epidemiology, Cystic Fibrosis/complications, Cystic Fibrosis Transmembrane Conductance Regulator, Ethnicity, Humans, Minority Groups, Oxygen, SARS-CoV-2",

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 Pierre-R{\'e}gis Burgel 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 {CF Registry Global Collaboration} and Geertjan Wesseling",

note = "Copyright {\textcopyright} 2022. Published by Elsevier B.V.",

year = "2022",

month = jul,

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 Science",

number = "4",

}

Carr, SB, McClenaghan, E, Elbert, A, Faro, A, Cosgriff, R, Abdrakhmanov, O, Brownlee, K, Burgel, P-R, 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, CF Registry Global Collaboration & Wesseling, G 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

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 - CF Registry Global Collaboration

AU - Wesseling, Geertjan

PY - 2022/7

Y1 - 2022/7

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 - COVID-19/epidemiology

KW - Cystic Fibrosis/complications

KW - Cystic Fibrosis Transmembrane Conductance Regulator

KW - Ethnicity

KW - Humans

KW - Minority Groups

KW - Oxygen

KW - SARS-CoV-2

U2 - 10.1016/j.jcf.2022.06.006

DO - 10.1016/j.jcf.2022.06.006

M3 - Article

C2 - 35753987

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

Keywords

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