Sars-CoV-2 infection in patients with cystic fibrosis. An overwiew

Main Article Content

Valentina Fainardi
Francesco Longo
Alfredo Chetta
Susanna Esposito
Giovanna Pisi


SARS-CoV-2, Cystic Fibrosis, Respiratory Viruses


The novel coronavirus SARS-CoV-2 was first identified in China in December 2019 and has since spread worldwide. People with Cystic Fibrosis (CF) have reduced survival mainly because of respiratory failure due to chronic pulmonary infections. Therefore, CF patients should be considered to have an increased risk of developing severe manifestations in case of SARS-CoV-2 infection. Surprisingly, the results of recent studies concerning SARS-CoV-2 infection in patients with CF show that in these patients the infection rate was lower than that of the general population. Various factors have been considered to explain a possible protective effect of CF against SARS-CoV-2 infection.


Download data is not yet available.


Metrics Loading ...
Abstract 75 | PDF Downloads 56


1. Guan W et al. Clinical Characteristics of Coronavirus Disease 2019 in China N Engl J Med 2020; 382:1708-1720.
2. World Health Organization. WHO Director- General’s opening remarks at the media briefing on COVID-19 -11 March 2020. WHO director- general- s- openingremarks-at- the- media- briefing- on- covid-19---11-march-2020
3. Fung SY et al. A tug-of-war between severe acute respiratory syndrome coronavirus 2 and host antiviral defence: lessons from other pathogenic viruses, Emerging Microbes & Infections 2020, 9, 558-570
4. Fehr AR, Perlman S. Coronaviruses: an overview of their replication and pathogenesis. Methods. Mol.Biol. 2015; 1282, 1–23).
5. Hoffmann et al. SARS-CoV-2 Cell Entry Depends on ACE2 and TMPRSS2 and Is Blocked by a Clinically Proven Protease Inhibitor. Cell, 2020, 181, 271–280).
6. Milanetti E, Miotto M, Di Rienzo L, Monti M, Gosti G, Ruocco G. In-Silico evidence for two receptors based strategy of SARS-CoV-2 bioRxiv preprint doi:
7. Tay MZ, Poh CM, Rénia L, MacAry PA, Ng LFP. The trinity of COVID-19: immunity, inflammation and intervention. Nature Reviews 2020, 20, 363-374.
8. Mehta P, McAuley DF, Brown M, et al. COVID-19: consider cytokine storm syndromes and immunosuppression. Lancet. 2020;395(10229):1033-1034.
9. Zhou F, Yu T, Du R, et al. Clinical course and risk factors for mortality of adult inpatients with COVID-19 in Wuhan, China: a retrospective cohort study. Lancet. 2020;395(10229):1054-1062).
10. Immovilli et al. COVID-19 mortality and ICU admission: the Italian experience Critical Care 2020, 24:228
11. Remuzzi A, Remuzzi G. COVID-19 and Italy: what next? Lancet 2020, 395: 1225–28
12. Garozzino S et al, Multicentre Italian study of SARS-CoV-2 infection in children and adolescents, preliminary data as at 10 April 2020. Euro Surveill. 2020 ; 25(18).
13. Elborn JS. Cystic fibrosis. Lancet. 2016; 388:2519-2531.
14. Ratjen F, Bell SC, Rowe SM, Goss CH, Quittner AL, and Bush A. Cystic fibrosis. Nat Rev Dis Primers 1: 15010, 2015
15. Keogh RH, Szczesniak R, Taylor-Robinson D, Bilton D. Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data. Journal of Cystic Fibrosis 2018; 17:218–227.
16. Cohen TS, Prince A. Cystic fibrosis: a mucosal immunodeficiency syndrome. Nat Med 2012, 18( 4), 510-519
17. Bruscia EM and Bonfield TL. Cystic Fibrosis Lung Immunity: The Role of the Macrophage J Innate Immun 2016; 8: 550-563
18. Skopelja S, Hamilton BJ, Jones JD, Yang ML, Mamula M, Ashare A, Gifford AH, and Rigby WF. The role for neutrophil extracellular traps in cystic fibrosis autoimmunity. JCI Insight 2016 1(17): e88912)
19. Cantin AM, Dominik Hartl D, Michael W. Konstan MW, James F. Chmiel JF. Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy. Journal of Cystic Fibrosis 2015; 14:419-430.
20. Stenbit AE, Flume PA. Pulmonary exacerbations in cystic fibrosis Curr opin Pulm Med 2011, 17(6), 442-447.
21. Wat D, Gelder C, Hibbitts S, Cafferty F, Bowler I, Pierrepoint M, Evans R, Doull I. The role of respiratory viruses in cystic fibrosis Journal of Cystic Fibrosis 2008; 7:320–328.
22. Viviani L, Assael BM, Kerem E. Impact of the A (H1N1) pandemic influenza (season 2009-2010) on patients with cystic fibrosis. J Cyst Fibros. 2011; 10:370–6
23. Somayaji R, Goss CH, Khan U, Neradilek M, Neuzil KM, and Ortiz JR. Cystic Fibrosis Pulmonary Exacerbations Attributable to Respiratory Syncytial Virus and Influenza: A Population-Based Study. Clin Infect Dis 2017; 64:1760-1767.
24. Chattoraj SS, Ganesan S, Faris A, Comstock A, Lee WM, and Sajjan US. Pseudomonas aeruginosa suppresses interferon response to rhinovirus infection in cystic fibrosis but not in normal bronchial epithelial cells. Infect Immun 2011; 79:4131-4145
25. Ramsey BW, Gore EJ, Smith AL, Cooney MK, Redding GJ, Foy H. The effect of respiratory viral infections on patients with cystic fibrosis. Am J Dis Child 1989; 143:662–8
26. Smyth AR, Smyth RL, Tong CY, Hart CA, Heaf DP. Effect of respiratory virus infections including rhinovirus on clinical status in cystic fibrosis. Arch Dis Child 1995;73(2):117–20.
27. Cosgriff R, Ahern S, Bell SC, et al. A multinational report to characterise SARS-CoV-2 infection in people with cystic fibrosis. Journal of Cystic Fibrosis 2020; 19: 355–358
28. Colombo C, Burgel P-R, Gartner S, et al. Impact of COVID-19 on people with cystic fibrosis. Lancet Respir Med 2020; 15 S2213-2600(20)30177-6
29. Yang X, Xu J, Shu H, Xia J, Liu H, Wu Y, et al. Clinical course and out- comes of critically ill patients with SARS-CoV-2 pneumonia in Wuhan, Chine: a single-centred, retrospective, observational study. Lancet Respir Med 2020 Feb 21 S2213-2600(20)30079-5.
31. Cigana C, Nicolis E, Pasetto M, Assael BM , Melotti P. Anti-inflammatory effects of azithromycin in cystic fibrosis airway epithelial cells. Biochemical and Biophysical Research Communications 2006; 350: 977–982
32. Menzel M, Akbarshahi H, Bjermer L, and Uller L. Azithromycin induces anti-viral effects in cultured bronchial epithelial cells from COPD patients. Sci Rep 2016; 6: 28698
33. Voisin G, Bouvet GF, Legendre P, Dagenais A, Masse C, and Berthiaume Y. Oxidative stress modulates the expression of genes involved in cell survival in DeltaF508 cystic fibrosis airway epithelial cells. Physiol Genomics 2014; 46: 634-646