RT Journal Article
SR Electronic
T1 Factors for severe outcomes following SARS-CoV-2 infection in people with cystic fibrosis in Europe
JF ERJ Open Research
JO erjor
FD European Respiratory Society
SP 00411-2021
DO 10.1183/23120541.00411-2021
A1 Jung, Andreas
A1 Orenti, Annalisa
A1 Dunlevy, Fiona
A1 Aleksejeva, Elina
A1 Bakkeheim, Egil
A1 Bobrovnichy, Vladimir
A1 Carr, Siobhán B.
A1 Colombo, Carla
A1 Corvol, Harriet
A1 Cosgriff, Rebecca
A1 Daneau, Géraldine
A1 Dogru, Deniz
A1 Drevinek, Pavel
A1 Vukic, Andrea Dugac
A1 Fajac, Isabelle
A1 Fox, Alice
A1 Fustik, Stojka
A1 Gulmans, Vincent
A1 Harutyunyan, Satenik
A1 Hatziagorou, Elpis
A1 Kasmi, Irena
A1 Kayserová, Hana
A1 Kondratyeva, Elena
A1 Krivec, Uroš
A1 Makukh, Halyna
A1 Malakauskas, Kestutis
A1 McKone, Edward F.
A1 Mei-Zahav, Meir
A1 de Monestrol, Isabelle
A1 Olesen, Hanne Vebert
A1 Padoan, Rita
A1 Parulava, Tsitsino
A1 Pastor-Vivero, Maria Dolores
A1 Pereira, Luísa
A1 Petrova, Guergana
A1 Pfleger, Andreas
A1 Pop, Liviu
A1 van Rens, Jacqui G.
A1 Rodić, Milan
A1 Schlesser, Marc
A1 Storms, Valérie
A1 Turcu, Oxana
A1 Woźniacki, Lukasz
A1 Yiallouros, Panayiotis
A1 Zolin, Anna
A1 Downey, Damian G.
A1 Naehrlich, Lutz
YR 2021
UL http://openres.ersjournals.com/content/early/2021/10/21/23120541.00411-2021.abstract
AB 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.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.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.