TY - JOUR
T1 - Factors for severe outcomes following SARS-CoV-2 infection in people with cystic fibrosis in Europe
AU - Jung, Andreas
AU - Orenti, Annalisa
AU - Dunlevy, Fiona
AU - Aleksejeva, Elina
AU - Bakkeheim, Egil
AU - Bobrovnichy, Vladimir
AU - Carr, Siobhán B.
AU - Colombo, Carla
AU - Corvol, Harriet
AU - Cosgriff, Rebecca
AU - Daneau, Géraldine
AU - Dogru, Deniz
AU - Drevinek, Pavel
AU - Vukic, Andrea Dugac
AU - Fajac, Isabelle
AU - Fox, Alice
AU - Fustik, Stojka
AU - Gulmans, Vincent
AU - Harutyunyan, Satenik
AU - Hatziagorou, Elpis
AU - Kasmi, Irena
AU - Kayserová, Hana
AU - Kondratyeva, Elena
AU - Krivec, Uroš
AU - Makukh, Halyna
AU - Malakauskas, Kestutis
AU - McKone, Edward F.
AU - Mei-Zahav, Meir
AU - de Monestrol, Isabelle
AU - Olesen, Hanne Vebert
AU - Padoan, Rita
AU - Parulava, Tsitsino
AU - Pastor-Vivero, Maria Dolores
AU - Pereira, Luísa
AU - Petrova, Guergana
AU - Pfleger, Andreas
AU - Pop, Liviu
AU - van Rens, Jacqui G.
AU - Rodić, Milan
AU - Schlesser, Marc
AU - Storms, Valérie
AU - Turcu, Oxana
AU - Woźniacki, Lukasz
AU - Yiallouros, Panayiotis
AU - Zolin, Anna
AU - Downey, Damian G.
AU - Naehrlich, Lutz
N1 - Funding Information:
Support statement: This paper was supported by an unrestricted grant from Chiesi Pharmaceuticals, Italy. The funder had no role in the planning, conduct, analysis or reporting of the study, nor did they review the draft paper before submission. Funding information for this article has been deposited with the Crossref Funder Registry.
Publisher Copyright:
© The authors 2021.
PY - 2021/10/1
Y1 - 2021/10/1
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85123306050&partnerID=8YFLogxK
U2 - 10.1183/23120541.00411-2021
DO - 10.1183/23120541.00411-2021
M3 - Article
AN - SCOPUS:85123306050
SN - 2312-0541
VL - 7
JO - ERJ Open Research
JF - ERJ Open Research
IS - 4
M1 - 00411-2021
ER -
