Factors for severe outcomes following SARS-CoV-2 infection in people with cystic fibrosis in Europe

Andreas Jung*, Annalisa Orenti, Fiona Dunlevy, Elina Aleksejeva, Egil Bakkeheim, Vladimir Bobrovnichy, Siobhán B. Carr, Carla Colombo, Harriet Corvol, Rebecca Cosgriff, Géraldine Daneau, Deniz Dogru, Pavel Drevinek, Andrea Dugac Vukic, Isabelle Fajac, Alice Fox, Stojka Fustik, Vincent Gulmans, Satenik Harutyunyan, Elpis HatziagorouIrena Kasmi, Hana Kayserová, Elena Kondratyeva, Uroš Krivec, Halyna Makukh, Kestutis Malakauskas, Edward F. McKone, Meir Mei-Zahav, Isabelle de Monestrol, Hanne Vebert Olesen, Rita Padoan, Tsitsino Parulava, Maria Dolores Pastor-Vivero, Luísa Pereira, Guergana Petrova, Andreas Pfleger, Liviu Pop, Jacqui G. van Rens, Milan Rodić, Marc Schlesser, Valérie Storms, Oxana Turcu, Lukasz Woźniacki, Panayiotis Yiallouros, Anna Zolin, Damian G. Downey, Lutz Naehrlich

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


Background Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in people with cystic fibrosis ( 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 December 31, 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% of pwCF were admitted to hospital, 2.5% of those to intensive care, and regretfully 11 (1.4%) died. Hospitalisation, oxygen therapy, intensive care, respiratory support and death were 2-to 6-fold more frequent in lung-transplanted versus non-lung-transplanted pwCF. Factors associated with hospitalisation and oxygen therapy were lung transplantation, cystic fibrosis-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 1 s <70% predicted, CFRD and those with lung transplants are at particular risk of more severe outcomes.

Original languageEnglish
Article number00411-2021
JournalERJ Open Research
Issue number4
StatePublished - 1 Oct 2021


FundersFunder number
Chiesi Farmaceutici


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