Abstract
BackgroundSevere acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and the development of life-threatening COVID-19 are believed to disproportionately affect certain at-risk populations. However, it is not clear whether individuals with cystic fibrosis (CF) are at a higher risk of COVID-19 or its adverse consequences. Recurrent respiratory viral infections are often associated with perturbation and pulmonary exacerbations of CF as evidenced by the significant morbidity observed in CF individuals during the 2009 H1N1 pandemic. The primary goal of this review was to systematically survey published accounts of COVID-19 in CF and determine if individuals with CF are disproportionally affected by SARS-CoV-2 and development of COVID-19.MethodsWe conducted a systematic literature search using EMBASE and Medline between April 28 and December 10, 2020. Six evaluable studies reporting on a total of 339 individuals with CF who developed COVID-19 were included in this study.ResultsWe found that although individuals with CF generally experience acute exacerbations of lung disease from infectious agents, COVID-19 incidence estimates in CF appear to be lower than in the general population. However, there are reports of subsets of CF, such as those who had organ transplants, that may experience a more severe COVID-19 course. Potential protective mechanisms in the CF population include pre-pandemic social isolation practices, infection prevention and control knowledge, altered expression of angiotensin-converting enzyme, and the use of certain medications.ConclusionsAlthough individuals with CF are at risk of acute exacerbations often precipitated by respiratory tract viral infections, published evidence to date indicated that individuals with CF do not experience higher risks of contracting SARS-CoV-2 infection. However, there is evidence that some subsets within the CF population, including those post-transplantation, may experience a more severe clinical course. As SARS-CoV-2 variants are identified and the pandemic goes through additional waves of disease outbreaks, ongoing monitoring of the risk of COVID-19 in individuals with CF is required.
Highlights
Cystic fibrosis (CF) is a classic Mendelian autosomal recessive disorder and is the most common fatal genetic disease in North America [1]
Information collected by the European Cystic Fibrosis Society Patient Registry (ECFSPR) on SARS-CoV-2 infection in 1236 individuals with cystic fibrosis (CF) from 30 countries was included [17]
The median age of the infected cohort was 33 years, which was higher than the median age of the general CF population, with only one patient (3%) under the age of 16; this is reflective of the lower reportage of pediatric cases of COVID-19 diagnosed in the non-CF
Summary
Cystic fibrosis (CF) is a classic Mendelian autosomal recessive disorder and is the most common fatal genetic disease in North America [1]. The predominant incidence estimate of the disorder is 1/2500 live births in Caucasians, with a mean prevalence of 0.797/10,000 in the United States of America [2, 3]. Abnormal CFTR are associated with aberrant salt and water transport across epithelial surfaces. Within the lungs, this manifests with mucus accumulation and the Mathew et al BMC Pulm Med (2021) 21:173. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and the development of life-threatening COVID-19 are believed to disproportionately affect certain at-risk populations. It is not clear whether individuals with cystic fibrosis (CF) are at a higher risk of COVID-19 or its adverse consequences. The primary goal of this review was to systematically survey published accounts of COVID-19 in CF and determine if individuals with CF are disproportionally affected by SARS-CoV-2 and development of COVID-19
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
