Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study.


Journal

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
ISSN: 1873-5010
Titre abrégé: J Cyst Fibros
Pays: Netherlands
ID NLM: 101128966

Informations de publication

Date de publication:
07 2022
Historique:
received: 01 03 2022
revised: 19 05 2022
accepted: 11 06 2022
pubmed: 27 6 2022
medline: 28 7 2022
entrez: 26 6 2022
Statut: ppublish

Résumé

This international study aimed to characterise the impact of acute SARS-CoV-2 infection in people with cystic fibrosis and investigate factors associated with severe outcomes. Methods Data from 22 countries prior to 13 SARS-CoV-2 was reported in 1555 people with CF, 1452 were included in the analysis. One third were aged <18 years, and 9.4% were solid-organ transplant recipients. 74.5% were symptomatic and 22% were admitted to hospital. In the non-transplanted cohort, 39.5% of patients with ppFEV1<40% were hospitalised with oxygen verses 3.2% with ppFEV >70%: a 17-fold increase in odds. Worse outcomes were independently associated with older age, non-white race, underweight body mass index, and CF-related diabetes. Prescription of highly effective CFTR modulator therapies was associated with a significantly reduced odds of being hospitalised with oxygen (AOR 0.43 95%CI 0.31-0.60 p<0.001). Transplanted patients were hospitalised with supplemental oxygen therapy (21.9%) more often than non-transplanted (8.8%) and was independently associated with the primary outcome (Adjusted OR 2.45 95%CI 1.27-4.71 p=0.007). This is the first study to show that there is a protective effect from the use of CFTR modulator therapy and that people with CF from an ethnic minority are at more risk of severe infection with SARS-CoV-2.

Sections du résumé

BACKGROUND
This international study aimed to characterise the impact of acute SARS-CoV-2 infection in people with cystic fibrosis and investigate factors associated with severe outcomes. Methods Data from 22 countries prior to 13
RESULTS
SARS-CoV-2 was reported in 1555 people with CF, 1452 were included in the analysis. One third were aged <18 years, and 9.4% were solid-organ transplant recipients. 74.5% were symptomatic and 22% were admitted to hospital. In the non-transplanted cohort, 39.5% of patients with ppFEV1<40% were hospitalised with oxygen verses 3.2% with ppFEV >70%: a 17-fold increase in odds. Worse outcomes were independently associated with older age, non-white race, underweight body mass index, and CF-related diabetes. Prescription of highly effective CFTR modulator therapies was associated with a significantly reduced odds of being hospitalised with oxygen (AOR 0.43 95%CI 0.31-0.60 p<0.001). Transplanted patients were hospitalised with supplemental oxygen therapy (21.9%) more often than non-transplanted (8.8%) and was independently associated with the primary outcome (Adjusted OR 2.45 95%CI 1.27-4.71 p=0.007).
CONCLUSIONS
This is the first study to show that there is a protective effect from the use of CFTR modulator therapy and that people with CF from an ethnic minority are at more risk of severe infection with SARS-CoV-2.

Identifiants

pubmed: 35753987
pii: S1569-1993(22)00593-8
doi: 10.1016/j.jcf.2022.06.006
pmc: PMC9189103
pii:
doi:

Substances chimiques

Cystic Fibrosis Transmembrane Conductance Regulator 126880-72-6
Oxygen S88TT14065

Types de publication

Journal Article Observational Study Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

e221-e231

Subventions

Organisme : CIHR
Pays : Canada

Investigateurs

Scott C Bell (SC)
David Reid (D)
Peter Wark (P)
Eva Van Braeckel (E)
Sophie Gohy (S)
Christiane Knoop (C)
Jessica Pirson (J)
Elke De Wachter (E)
Lieven Dupont (L)
Laurence Hanssens (L)
Vicky Nowé (V)
Monique Lequesne (M)
Rodrigo A Athanazio (RA)
Daniela G Meneses (DG)
Véronique Boussaud (V)
Graziella Brinchault (G)
Emmanuelle Coirier-Duet (E)
Jean-Christophe Dubus (JC)
Dominique Grenet (D)
Sandra de Miranda (S)
Laurence Beaumont (L)
Reem Kanaan (R)
Muriel Lauraens (M)
Clémence Martin (C)
Marie Mittaine (M)
Anne Prévotat (A)
Martine Reynaud-Gaubert (M)
Isabelle Sermet-Gaudelus (I)
Aurelie Tatopoulos (A)
Raphael Chiron (R)
Marie-Laure Dalphin (ML)
Michele Gerardin (M)
Laurence Weiss (L)
Nathalie Wizla (N)
Sophie Ramel (S)
Barry Plant (B)
Cedric Gunaratnam (C)
Abaigeal Jackson (A)
Karin de Winter-de Groot (K)
Bart Luijk (B)
Geertjan Wesseling (G)
Elena Kondratyeva (E)
Elena Zhekayte (E)
Elena Amelina (E)
Mariya Mukhina (M)
Olga Simonova (O)
Antonio Alvarez-Fernandez (A)
Amparo Sole-Jover (A)
Isidoro Cortell-Aznar (I)
Rosa Girón-Moreno (R)
Alejandro López-Neyra (A)
Isabel Ramos-Cancelo (I)
Maite Lázaro-Carrasco (M)
Dolores Pastor Vivero (DP)
Marta Ruiz de Valbuena (MR)
Concepción Prados-Sanchez (C)
Jordi Costa-Colomer (J)
Silvia Gartner (S)
Layla Diab-Caceres (L)
Marita Gilljam (M)
Ulrika Lindberg (U)
Stefanie Diemer (S)
Mark Allenby (M)
Stephen J Bourke (SJ)
Susan C Charman (SC)
Janet Collinson (J)
Owen Dempsey (O)
Sarah Denniston (S)
Maya Desai (M)
Jamie Duckers (J)
Christine Etherington (C)
Elaine Gunn (E)
Alex Higton (A)
Timothy Ho (T)
Jeremy Hull (J)
Andrew Jones (A)
Robert Ian Ketchell (RI)
Susan L Madge (SL)
Anirban Maitra (A)
Ghulam Mujtaba (G)
Edward Nash (E)
Dilip Nazareth (D)
Christopher O'Brien (C)
Claire Onyon (C)
Christopher Orchard (C)
Daniel Peckham (D)
Helen Rodgers (H)
Nadia Shafi (N)
Nicholas Simmonds (N)
Kevin Southern (K)
Martin Walshaw (M)
Danie Watson (D)
Joanna L Whitehouse (JL)

Informations de copyright

Copyright © 2022. Published by Elsevier B.V.

Déclaration de conflit d'intérêts

COI Statement All authors declare no conflicts of interest in relationship to this work. Outside of this work the following authors declare payments or honoraria to them or their institution for a combination of lectures, presentations, educational events, advisory boards, steering groups, grants or consultancy fees: SC-Vertex, Chiesi, Profile, Zambon. RC- Vertex, P-RB – Astra-Zeneca, Boehringer Ingelheim, GSK, Insmed, Chiesi, Pfizer, Vertex, Zambon. I deM – Vertex,LN – Vertex, Boehringer,LVS-F – Vertex, AS -Vertex.

Auteurs

Siobhán B Carr (SB)

Royal Brompton Hospital, part of GSST NHS Foundation Trust, London, UK; NHLI, Imperial College, London, UK. Electronic address: s.carr@rbht.nhs.uk.

Elliot McClenaghan (E)

Cystic Fibrosis Trust, London, UK; London School of Hygiene and Tropical Medicine, London, UK.

Alexander Elbert (A)

Cystic Fibrosis Foundation, USA.

Albert Faro (A)

Cystic Fibrosis Foundation, USA.

Rebecca Cosgriff (R)

Cystic Fibrosis Trust, London, UK.

Olzhas Abdrakhmanov (O)

The Second Children's Hospital, Kazakhstan.

Keith Brownlee (K)

Cystic Fibrosis Trust, London, UK.

Pierre-Régis Burgel (PR)

Université de Paris, Inserm U1016, Institut Cochin and Cochin Hospital, Assistance Publique Hôpitaux de Paris (APHP), France.

Catherine A Byrnes (CA)

Starship Children's Hospital and University of Auckland, Auckland, New Zealand.

Stephanie Y Cheng (SY)

Cystic Fibrosis, Canada.

Carla Colombo (C)

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan, Italy.

Harriet Corvol (H)

Sorbonne Université, Inserm, Centre de Recherche Saint-Antoine, Assistance Publique Hôpitaux de Paris (APHP), Hôpital Trousseau, Service de Pneumologie Pédiatrique, Paris, France.

Géraldine Daneau (G)

Sciensano, Belgium.

Christopher H Goss (CH)

University of Washington, Seattle, USA.

Vincent Gulmans (V)

Dutch CF Foundation NCFS, Baarn, Netherlands.

Hector Gutierrez (H)

University of Alabama at Birmingham, Alabama, USA.

Satenik Harutyunyan (S)

Yerevan University CF Centre, Muratsan Hospital, Yerevan, Armenia.

Meagan Helmick (M)

Cystic Fibrosis Foundation, USA.

Andreas Jung (A)

University Children's Hospital, Zurich, Switzerland.

Nataliya Kashirskaya (N)

Research Centre for Medical Genetics, Russia.

Edward McKone (E)

St Vincent's University Hospital, Dublin, Ireland.

Joel Melo (J)

Instituto Nacional Del Torax, Chile.

Peter G Middleton (PG)

Westmead Hospital, Sydney, Australia.

Pedro Mondejar-Lopez (P)

Hospital Clínico Universitario Virgen de la Arrixaca, Murcia, Spain.

Isabelle de Monestrol (I)

Department of Pediatrics, CLINTEC, Karolinska Institutet; Karolinska University Hospital Huddinge, Sweden.

Lutz Nährlich (L)

Department of Pediatrics, Justus-Liebig-University Giessen, Giessen, Germany.

Rita Padoan (R)

Cystic Fibrosis Support Center, Department of Paediatric, University of Brescia, Italy.

Megan Parker (M)

Cystic Fibrosis, Canada.

M Dolores Pastor-Vivero (MD)

Hospital Universitario Cruces, IIC Biocruces-Bizkaia, Bizkaia.

Samar Rizvi (S)

Cystic Fibrosis Foundation, USA.

Rasa Ruseckaite (R)

Monash University, Melbourne, Australia.

Marco Salvatore (M)

Istituto Superiore di Sanita, National Center Rare Diseases, Undiagnosed Rare Diseases Interdepartmental Unit, Rome, Italy.

Luiz Vicente R F da Silva-Filho (LVRF)

Instituto da Criança HCFMUSP, São Paulo, Brazil.

Nick Versmessen (N)

Expert by Experience, Ghent University, Belgium.

Marco Zampoli (M)

University of Cape Town and Red Cross War Memorial Children's Hospital, South Africa.

Bruce C Marshall (BC)

Cystic Fibrosis Foundation, USA.

Anne L Stephenson (AL)

Cystic Fibrosis, Canada; Toronto Adult Cystic Fibrosis Centre, St. Michael's Hospital, Toronto, Canada.

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Classifications MeSH