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Table 3 Potential CFTR Modulators positive effects in individuals with Cystic Fibrosis infected with SARS-CoV-2

From: The impact of SARS-CoV-2 infection in patients with cystic fibrosis undergoing CFTR channel modulators treatment: a literature review

CFTR modulators effect

References

Anti-inflammatory and significant reduction inflammatory mediators

Hisert KB et al. Restoring cystic fibrosis transmembrane conductance regulator function reduces airway bacteria and inflammation in people with cystic fibrosis and chronic lung infections. Am J Respir Crit CareMed. 2017; 195(12): 1617- 1628. https://doi.org/10.1164/rccm.201609-1954OC [61]

Improve airways mucus clearance

Altes TA et al. Use of hyperpolarized helium-3 MRI to assess response to ivacaftor treatment in patients with cystic fibrosis. J Cyst Fibros. 2017; 16(2): 267- 274. https://doi.org/10.1016/j.jcf.2016.12.004 [62]

Inhibition in vitro SARS-CoV-2 replication

Lotti V et al. CFTR Modulation Reduces SARS-CoV-2 Infection in Human Bronchial Epithelial Cells. Cells. 2022;11:1347. https://doi.org/10.3390/cells11081347 [63]

Significantly improved ppFEV1

Quittner AL et al. Development and validation of The Cystic Fibrosis Questionnaire in the United States: a health-related quality-of-life measure for cystic fibrosis. Chest 2005 Oct;128(4):2347–54. https://doi.org/10.1378/chest.128.4.2347 [46]

Significant improvements in respiratory function

Wainwright CE et al. Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N. Engl. J. Med. 373:220–31. https://doi.org/10.1056/NEJMoa1409547 [47]