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Observational Study
. 2022 Jul;21(4):e221-e231.
doi: 10.1016/j.jcf.2022.06.006. Epub 2022 Jun 13.

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

Siobhán B Carr  1 , Elliot McClenaghan  2 , Alexander Elbert  3 , Albert Faro  3 , Rebecca Cosgriff  4 , Olzhas Abdrakhmanov  5 , Keith Brownlee  4 , Pierre-Régis Burgel  6 , Catherine A Byrnes  7 , Stephanie Y Cheng  8 , Carla Colombo  9 , Harriet Corvol  10 , Géraldine Daneau  11 , Christopher H Goss  12 , Vincent Gulmans  13 , Hector Gutierrez  14 , Satenik Harutyunyan  15 , Meagan Helmick  3 , Andreas Jung  16 , Nataliya Kashirskaya  17 , Edward McKone  18 , Joel Melo  19 , Peter G Middleton  20 , Pedro Mondejar-Lopez  21 , Isabelle de Monestrol  22 , Lutz Nährlich  23 , Rita Padoan  24 , Megan Parker  8 , M Dolores Pastor-Vivero  25 , Samar Rizvi  3 , Rasa Ruseckaite  26 , Marco Salvatore  27 , Luiz Vicente R F da Silva-Filho  28 , Nick Versmessen  29 , Marco Zampoli  30 , Bruce C Marshall  3 , Anne L Stephenson  31 ; CF Registry Global Collaboration
Collaborators, Affiliations
Observational Study

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

Siobhán B Carr et al. J Cyst Fibros. 2022 Jul.

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.

Keywords: COVID-19; Coronavirus; Cystic fibrosis; SARS-CoV-2; Transplant.

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Conflict of interest statement

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.

Figures

Fig 1
Fig. 1
Study flow diagram.
Fig 2
Fig. 2
Disease course by transplant status and baseline Best ppFEV1. People >6 years, with non-missing outcome and baseline best ppFEV1 data are included in this graph (N=1047). Total non-missing for each group are represented as bar labels – e.g., N=81 non-transplanted patients with <40% ppFEV1 had non-missing outcome values. Outcomes are coded as mutually exclusive – note that this differs from how the data are presented in Table 2, Table 3.
Fig 3
Fig. 3
Multivariable analysis for hospitalisation with supplemental oxygen in all patients (n=1362, m10). All analyses are adjusted for clustering on country using robust standard errors. Excluding people aged <6 years as ppFEV1 measurements are not recommended in this age group. p values shown represent the overall p values for the variable and are not associated with the level-to-level comparisons within these variables, which are represented by the 95% CIs. CI=Confidence interval ppFEV1=forced expiratory volume in 1 second percent predicted, BMI=body mass index, Iva=ivacaftor, elex=elexacaftor, tez=tezacaftor m10=multiple imputed dataset with 10 iterations.
Fig 4
Fig. 4
Multivariable analysis for hospitalisation with supplemental oxygen in the non-transplant cohort (N=1226, m=10). All analyses are adjusted for clustering on country using robust standard errors. Excluding people aged <6 years as ppFEV1 measurements are not recommended in this age group. p values shown represent the overall p values for the variable and are not associated with the level-to-level comparisons within these variables, which are represented by the 95% CIs. CI=Confidence interval ppFEV1=forced expiratory volume in 1 second percent predicted, BMI=body mass index, Iva=ivacaftor, elex=elexacaftor, tez=tezacaftor m10=multiple imputed dataset with 10 iterations.
See this image and copyright information in PMC

Comment in

  • The use of dornase alfa in patients with COVID-19.
    Ramachandram DS, Kow CS, Hasan SS. Ramachandram DS, et al. J Cyst Fibros. 2023 May;22(3):580. doi: 10.1016/j.jcf.2023.03.010. Epub 2023 Mar 13. J Cyst Fibros. 2023. PMID: 36966036 Free PMC article. No abstract available.

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