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

Abstract

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 13th December 2020 and the introduction of vaccines were included. It was de-identified and included patient demographics, clinical characteristics, treatments, outcomes and sequalae following SARS-CoV-2 infection. Multivariable logistic regression was used to investigate factors associated with clinical progression to severe COVID-19, using the primary outcome of hospitalisation with supplemental oxygen.

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.

• Authors: Siobhán B Carr, Elliot McClenaghan, Alexander Elbert, Albert Faro, Rebecca Cosgriff, Olzhas Abdrakhmanov, Keith Brownlee, Pierre-Régis Burgel, Catherine A Byrnes, Stephanie Y Cheng, Carla Colombo, Harriet Corvol, Géraldine Daneau, Christopher H Goss, Vincent Gulmans, Hector Gutierrez, Satenik Harutyunyan, Meagan Helmick, Andreas Jung, Nataliya Kashirskaya, Edward McKone, Joel Melo, Peter G Middleton, Pedro Mondejar-Lopez, Isabelle de Monestrol, Lutz Nährlich, Rita Padoan, Megan Parker, M Dolores Pastor-Vivero, Samar Rizvi, Rasa Ruseckaite, Marco Salvatore, Luiz Vicente R F da Silva-Filho, Nick Versmessen, Marco Zampoli, Bruce C Marshall, Anne L Stephenson, CF Registry Global Collaboration
• Journal: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society (J Cyst Fibros) Vol. 21 Issue 4 Pg. e221-e231 (07 2022) ISSN: 1873-5010 [Electronic] Netherlands
• PMID: 35753987 (Publication Type: Journal Article, Observational Study, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2022. Published by Elsevier B.V.

Chemical References

• Cystic Fibrosis Transmembrane Conductance Regulator
• Oxygen

Topics

• COVID-19 (epidemiology, therapy)
• Cystic Fibrosis (complications, epidemiology, therapy)
• Cystic Fibrosis Transmembrane Conductance Regulator
• Ethnicity
• Humans
• Minority Groups
• Oxygen
• SARS-CoV-2