COVID-19 Evidence Alerts
from McMaster PLUSTM

Current best evidence for clinical care (more info)

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Prognosis Mathew HR, Choi MY, Parkins MD, et al. Systematic review: cystic fibrosis in the SARS-CoV-2/COVID-19 pandemic. BMC Pulm Med. 2021 May 20;21(1):173. doi: 10.1186/s12890-021-01528-0.

BACKGROUND: 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. 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.

METHODS: We 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.

RESULTS: We 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.

CONCLUSIONS: Although 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.

Discipline / Specialty Area Score
Pediatric Hospital Medicine
Pediatrics (General)
Intensivist/Critical Care
Infectious Disease
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Comments from MORE raters

Pediatric Hospital Medicine rater

This systematic review of observational studies identifies the clinical evolution of patients with cystic fibrosis who become ill with COVID-19. Identifying a lower incidence and lower severity, the document emphasizes different clinical courses in previously transplanted patients. Preventive measures against COVID-19 are not an exception in this pediatric population, and the observations derived from this study do not change the recommendations.

Pediatric Hospital Medicine rater

This fits with clinician observation.

Respirology/Pulmonology rater

This is a useful overview. It is to be assumed that CF patients would be much more cautious in a Covid-19 environment so the incidence rates are not unexpected. However it is reassuring that, apart from some subgroups, the CF patients overall did not have poor outcomes if they became infected.