Abstract
Background SARS-Co-V-2 infection in people with CF (pwCF) can lead to severe outcomes.
Methods In this observational study, the European Cystic Fibrosis Society Patient Registry collected data on pwCF and SARS-CoV-2 infection to estimate incidence, describe clinical presentation and investigate factors associated with severe outcomes using multivariable analysis.
Results Up to 31 December 2020, 26 countries reported information on 828 pwCF and SARS-CoV-2 infection. Incidence was 17.2 per 1000 pwCF (95% CI: 16.0–18.4). Median age was 24 years, 48.4% were male and 9.4% had lung transplants. SARS-CoV-2 incidence was higher in lung-transplanted (28.6 [95% CI: 22.7–35.5]) versus non-lung transplanted pwCF (16.6 [95% CI: 15.4–17.8]) (p=<0.001).
SARS-CoV-2 infection caused symptomatic illness in 75.7%. Factors associated with symptomatic SARS-CoV-2 infection were age >40 years, at least one F508del mutation, and pancreatic insufficiency.
Overall, 23.7% were admitted to hospital, 2.5% to intensive care. Regretfully 11 pwCF (1.4%) died. Hospitalisation, oxygen therapy, intensive care, respiratory support and death were 2–6-fold more frequent in lung-transplanted versus non-lung transplanted pwCF.
Factors associated with hospitalisation and oxygen therapy were lung transplantation, CF-related diabetes (CFRD), moderate or severe lung disease and azithromycin use (often considered a surrogate marker for Pseudomonas aeruginosa infection and poorer lung function).
Conclusion SARS-CoV-2 infection yielded high morbidity and hospitalisation in pwCF. PwCF with forced expiratory volume in one second (FEV1) <70% predicted, CFRD and those with lung transplants are at particular risk of more severe outcomes.
Footnotes
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Conflict of interest: Andreas Jung has nothing to disclose.
Conflict of interest: Annalisa Orenti has nothing to disclose.
Conflict of interest: Fiona Dunlevy reports support for the present manuscript from Chiesi Farmaceutici SpA.
Conflict of interest: Elina Aleksejeva has nothing to disclose.
Conflict of interest: Egil Bakkeheim has nothing to disclose.
Conflict of interest: Vladimir Bobrovnichy has nothing to disclose.
Conflict of interest: Siobhán B. Carr reports receiving speaker honoraria from Vertex and Chiesi, outside the submitted work. Participation on a Data Safety Monitoring Board or Advisory Board for Vertex, Profile Pharma and Chiesi, outside the submitted work. Leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid from UK CF Trust. PI for Pharmacovigilance study, payment to institution from Pharmaxis, outside the submitted work.
Conflict of interest: Carla Colombo has nothing to disclose.
Conflict of interest: Harriet Corvol has nothing to disclose.
Conflict of interest: Rebecca Cosgrif declares outside the submitted work to be the director of the Cystic Fibrosis Trust.
Conflict of interest: Géraldine Daneau has nothing to disclose.
Conflict of interest: Deniz Dogru has nothing to disclose.
Conflict of interest: Pavel Drevinek reports grants or contracts from Ministry of Health, Czech Republic, outside the submitted work. Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Vertex Pharmaceuticals and Actelion Pharmaceuticals, outside the submitted work. Participation on a Data Safety Monitoring Board or Advisory Board for Vertex Pharmaceuticals, outside the submitted work. Leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid from Society for Medical Microbiology, outside the submitted work.
Conflict of interest: Andrea Dugac Vukic has nothing to disclose.
Conflict of interest: Isabelle Fajac reports grants or contracts from Boehringer Ingelheim, Celtaxsys, Corbus Pharmaceuticals, and Vertex Pharmaceuticals, outside the submitted work. Consulting fees from Boehringer Ingelheim and Vertex Pharmaceuticals, outside the submitted work. Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Vertex Pharmaceuticals and Boehringer Ingelheim, outside the submitted work. Leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid for European Cystic Fibrosis Society.
Conflict of interest: Alice Fox support for the present manuscript from Chiesi Farmaceutici SpA.
Conflict of interest: Stojka Fustik has nothing to disclose.
Conflict of interest: Vincent Gulmans has nothing to disclose.
Conflict of interest: Satenik Harutyunyan has nothing to disclose.
Conflict of interest: Elpis Hatziagorou has nothing to disclose.
Conflict of interest: Irena Kasmi has nothing to disclose.
Conflict of interest: Hana Kayserová has nothing to disclose.
Conflict of interest: Elena Kondratyeva reports payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from All-Russian online school with international participation, outside the submitted work.
Conflict of interest: Uroš Krivec has nothing to disclose.
Conflict of interest: Halyna Makukh has nothing to disclose.
Conflict of interest: Kestutis Malakauskas has nothing to disclose.
Conflict of interest: Edward F. McKone reports grants or contracts from vertex, outside the submitted work. Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Vertex Pharmaceuticals and Roche Pharmaceuticals, outside the submitted work. Support for attending meetings and/or travel from A Menarini, outside the submitted work. Participation on a Data Safety Monitoring Board or Advisory Board for Insmed and Janssen Pharmaceuticals, outside the submitted work.
Conflict of interest: Meir Mei-Zahav has nothing to disclose.
Conflict of interest: Isabelle de Monestrol reports grant from Vertex for an academic research study regarding gastrointestinal outcome in CF patients taking Orkambi medication, outside the submitted work. Leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid from Swedish CF Registry and the Swedish Society of Medicine's CF working group.
Conflict of interest: Hanne Vebert Olesen has nothing to disclose.
Conflict of interest: Rita Padoan has nothing to disclose.
Conflict of interest: Tsitsino Parulava has nothing to disclose.
Conflict of interest: Maria Dolores Pastor-Vivero has nothing to disclose.
Conflict of interest: Luísa Pereira has nothing to disclose.
Conflict of interest: Guergana Petrova Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from TEVA Pharmaceuticals, Mylan and Alvogen, outside the submitted work.
Conflict of interest: Andreas Pfleger has nothing to disclose.
Conflict of interest: Liviu Pop has nothing to disclose.
Conflict of interest: Jacqui G. van Rens has nothing to disclose.
Conflict of interest: Milan Rodić has nothing to disclose.
Conflict of interest: Marc Schlesser has nothing to disclose.
Conflict of interest: Valérie Storms has nothing to disclose.
Conflict of interest: Oxana Turcu has nothing to disclose.
Conflict of interest: Lukasz Woźniacki has nothing to disclose.
Conflict of interest: Panayiotis Yiallouros has nothing to disclose.
Conflict of interest: Anna Zolin has nothing to disclose.
Conflict of interest: Damian G. Downey has nothing to disclose.
Conflict of interest: Lutz Naehrlich reports support for the present manuscript from Chiesi Farmaceutici SpA. Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from ArticulateScience LLC, outside the submitted work. Participation on a Data Safety Monitoring Board or Advisory Board for Trial Steering committee of CF Storm, outside the submitted work. Leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid from Society for German CF Registry European CF Patient Registry, outside the submitted work. Other financial or nonfinancial interests from Vertex Pharmaceuticals and Boehringer Ingelheim; Institutional fees for site participation (PI) in clinical trials from Vertex Pharmaceuticals and Boehringer Ingelheim.
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- Received June 21, 2021.
- Accepted September 12, 2021.
- Copyright ©The authors 2021
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