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. © 2022

• author: Carr, S.B. and Stephenson, Anne L.
• contributor: Diemer, S. and Lindberg, U.
• author collaboration: CF Registry Global Collaboration
• organization: Respiratory Medicine, Allergology, and Palliative Medicine; Paediatrics (Lund)
• publishing date: 2022
• type: Contribution to journal
• publication status: published
• subject: Family Medicine
• keywords: Coronavirus, COVID-19, Cystic fibrosis, SARS-CoV-2, Transplant, cystic fibrosis transmembrane conductance regulator, elexacaftor plus ivacaftor plus tezacaftor, ivacaftor, ivacaftor plus lumacaftor, ivacaftor plus tezacaftor, oxygen, SARS-CoV-2 vaccine, adult, all cause mortality, Article, artificial ventilation, body mass, cohort analysis, controlled study, coronavirus disease 2019, coughing, cystic fibrosis, demographics, disease exacerbation, drug efficacy, dyspnea, extracorporeal oxygenation, fatigue, female, fever, forced expiratory volume, genotype, graft recipient, hospital admission, hospital patient, hospitalization, human, hypertension, intensive care unit, intubation, invasive ventilation, male, myalgia, obesity, observational study, outcome assessment, oxygen therapy, pancreatic insufficiency, prescription, Pseudomonas aeruginosa, Pseudomonas infection, race, Severe acute respiratory syndrome coronavirus 2, underweight, complication, ethnicity, minority group, Cystic Fibrosis, Cystic Fibrosis Transmembrane Conductance Regulator, Ethnicity, Humans, Minority Groups, Oxygen
• in: Journal of Cystic Fibrosis
• volume: 21
• issue: 4
• pages: 221 - 231
• publisher: Elsevier
• external identifiers: scopus:85135282334; pmid:35753987
• ISSN: 1569-1993
• DOI: 10.1016/j.jcf.2022.06.006
• language: English
• LU publication?: yes
• id: a9a8d157-b67b-4a90-987e-0bb4d1e47841
• date added to LUP: 2022-09-16 11:59:27
• date last changed: 2023-09-09 07:03:58