ERJ Open Research (Dec 2021)
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 Hatziagorou,
- Irena 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 Rodic´,
- Marc Schlesser,
- Valérie Storms,
- Oxana Turcu,
- Lukasz Woz´niacki,
- Panayiotis Yiallouros,
- Anna Zolin,
- Damian G. Downey,
- Lutz Naehrlich
Affiliations
- Andreas Jung
- Paediatric Pulmonology, University Children's Hospital Zurich, Zurich, Switzerland
- Annalisa Orenti
- Dept of Clinical Sciences and Community Health, Laboratory of Medical Statistics, Epidemiology and Biometry G.A. Maccacaro, University of Milan, Milan, Italy
- Fiona Dunlevy
- European Cystic Fibrosis Society, Karup, Denmark
- Elina Aleksejeva
- Dept of Pneumology, Children's Clinical University Hospital, Rīga Stradinš University, Riga, Latvia
- Egil Bakkeheim
- Dept of Paediatrics, Norwegian Cystic Fibrosis Registry, Oslo University Hospital, Oslo, Norway
- Vladimir Bobrovnichy
- Belarusian Republic Children's Center of Pulmonology and Cystic Fibrosis, Pulmonary Department, 3rd City Children's Clinical Hospital, Minsk, Belarus
- Siobhán B. Carr
- Dept of Respiratory Paediatrics, Royal Brompton Hospital, London, UK
- Carla Colombo
- Dept of Pathophysiology and Transplantation, Cystic Fibrosis Reference Center of Lombardia Region, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan, Milan, Italy
- Harriet Corvol
- Pediatric Pulmonology Dept and Cystic Fibrosis Center, Sorbonne Université, Centre de Recherche Saint-Antoine, Inserm UMR_S938, Assistance Publique-Hôpitaux de Paris, Hôpital Trousseau, Paris, France
- Rebecca Cosgriff
- Cystic Fibrosis Trust, London, UK
- Géraldine Daneau
- Sciensano, Epidemiology and Public Health, Health Services Research, Brussels, Belgium
- Deniz Dogru
- Cystic Fibrosis Registry of Turkey, Ankara, Turkey
- Pavel Drevinek
- Dept of Medical Microbiology, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic
- Andrea Dugac Vukic
- University Hospital Centre Zagreb, Cystic Fibrosis Centre – Paediatrics and Adults, Zagreb, Croatia
- Isabelle Fajac
- Université Paris Descartes, Sorbonne Paris Cité, Paris, France
- Alice Fox
- European Cystic Fibrosis Society, Karup, Denmark
- Stojka Fustik
- Centre for Cystic Fibrosis, University Children's Hospital, Skopje, North Macedonia
- Vincent Gulmans
- Dutch Cystic Fibrosis Foundation (NCFS), Baarn, The Netherlands
- Satenik Harutyunyan
- Yerevan University CF Centre, Muratsan Hospital, Yerevan, Armenia
- Elpis Hatziagorou
- Cystic Fibrosis Unit, Hippokration General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
- Irena Kasmi
- Dept of Paediatrics, “Mother Thereza” Hospital Center, Tirana, Albania
- Hana Kayserová
- Cystic Fibrosis Centre, University Hospital of Bratislava, Bratislava, Slovakia
- Elena Kondratyeva
- Clinical Research Dept of Cystic Fibrosis “Research Centre for Medical Genetics”, Moscow, Russian Federation
- Uroš Krivec
- Dept of Paediatric Pulmonology, University Children's Hospital, Ljubljana University Medical Centre, Ljubljana, Slovenia
- Halyna Makukh
- Institute of Hereditary Pathology, Ukrainian National Academy of Medical Sciences, Lviv, Ukraine
- Kestutis Malakauskas
- Adult Cystic Fibrosis Center, Dept of Pulmonology, Lithuanian University of Health Sciences, Kaunas, Lithuania
- Edward F. McKone
- St Vincent's University Hospital & University College Dublin School of Medicine, Dublin, Ireland
- Meir Mei-Zahav
- Pulmonary Institute, Schneider Children's Medical Center of Israel, Petah Tikva, Israel
- Isabelle de Monestrol
- Stockholm CF Centre, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden
- Hanne Vebert Olesen
- Dept of Pediatrics and Adolescent Medicine, Cystic Fibrosis Center, Aarhus University Hospital, Aarhus, Denmark
- Rita Padoan
- Dept of Paediatrics, Cystic Fibrosis Regional Support Centre, University of Brescia, Brescia
- Tsitsino Parulava
- I. Tsitsishvili Children's Clinic, CF Centre, Tblisi, Georgia
- Maria Dolores Pastor-Vivero
- Pediatric Pneumology and Cystic Fibrosis Unit, Osakidetza, Hospital Universitario Cruces, Bizkaia, Spain
- Luísa Pereira
- Centre for Cystic Fibrosis, Hospital de Santa Maria, Lisbon, Portugal
- Guergana Petrova
- Pediatric Clinic, Alexandrovska University Hospital, Medical University, Sofia, Bulgaria
- Andreas Pfleger
- Dept of Pediatrics and Adolescent Medicine, Division of Pediatric Pulmonology and Allergology, Medical University of Graz, Graz, Austria
- Liviu Pop
- Victor Babes University of Medicine and Pharmacy Timisoara, National Cystic Fibrosis Centre, Timisoara, Romania
- Jacqui G. van Rens
- European Cystic Fibrosis Society, Karup, Denmark
- Milan Rodic´
- National Centre for Cystic Fibrosis, Mother and Child Health Institute of Serbia “Dr Vukan Čupić”, Belgrade, Serbia
- Marc Schlesser
- Dept of Pulmonology, Hôpital Robert Schuman, Luxembourg, Luxembourg
- Valérie Storms
- Cystic Fibrosis Europe, Brussels, Belgium
- Oxana Turcu
- Dept of Pediatrics, Ambulatory Cystic Fibrosis and Other Rare Diseases Center, Institute for Maternal and Child Healthcare, State University of Medicine and Pharmacy “Nicolae Testemitanu”, Chisinau, Republic of Moldova
- Lukasz Woz´niacki
- Dziekanow Paediatric Hospital, Cystic Fibrosis Centre, Institute of Mother and Child, Warsaw, Poland
- Panayiotis Yiallouros
- Medical School, University of Cyprus, Nicosia, Cyprus
- Anna Zolin
- Dept of Clinical Sciences and Community Health, Laboratory of Medical Statistics, Epidemiology and Biometry G.A. Maccacaro, University of Milan, Milan, Italy
- Damian G. Downey
- Wellcome–Wolfson Institute for Experimental Medicine, Queen's University Belfast, Belfast, UK
- Lutz Naehrlich
- Dept of Clinical Sciences and Community Health, Laboratory of Medical Statistics, Epidemiology and Biometry G.A. Maccacaro, University of Milan, Milan, Italy
- DOI
- https://doi.org/10.1183/23120541.00411-2021
- Journal volume & issue
-
Vol. 7,
no. 4
Abstract
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.
