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

Abstract Several risk factors for Coronavirus-2019 (COVID-19) disease have been highlighted in clinical evidence. Among the various risk factors are advanced age, metabolic illness such as diabetes, heart disease, and diseases of the respiratory system. Cystic Fibrosis (CF) is a rare disease with au...

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Main Authors: Antonio Vitiello, Michela Sabbatucci, Andrea Silenzi, Annalisa Capuano, Francesco Rossi, Andrea Zovi, Francesco Blasi, Giovanni Rezza
Format: Article
Language:English
Published: BMC 2023-11-01
Series:Respiratory Research
Subjects:
Online Access:https://doi.org/10.1186/s12931-023-02593-1
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author Antonio Vitiello
Michela Sabbatucci
Andrea Silenzi
Annalisa Capuano
Francesco Rossi
Andrea Zovi
Francesco Blasi
Giovanni Rezza
author_facet Antonio Vitiello
Michela Sabbatucci
Andrea Silenzi
Annalisa Capuano
Francesco Rossi
Andrea Zovi
Francesco Blasi
Giovanni Rezza
author_sort Antonio Vitiello
collection DOAJ
description Abstract Several risk factors for Coronavirus-2019 (COVID-19) disease have been highlighted in clinical evidence. Among the various risk factors are advanced age, metabolic illness such as diabetes, heart disease, and diseases of the respiratory system. Cystic Fibrosis (CF) is a rare disease with autosomal recessive transmission, characterised by a lack of synthesis of the CFTR channel protein, and multi-organ clinical symptoms mainly affecting the respiratory tract with recurrent pulmonary exacerbations. In view of the pathophysiological mechanisms, CF disease should be in theory considered a risk factor for SARS-CoV2 or severe COVID-19. However, recent clinical evidence seems to point in the opposite direction, suggesting that CF could be a protective factor against severe COVID-19. Possibly, the lack of presence or function of the CFTR channel protein could be linked to the expression of the membrane glycoprotein ACE-2, a key enzyme for the endocellular penetration of SARS-CoV-2 and related to the pathophysiology of COVID-19 disease. Furthermore, CFTR channel modulating agents could indirectly influence the expression of ACE-2, playing an important role in restoring the proper functioning of mucociliary clearance and the pulmonary microbiome in the host response to SARS-CoV-2 infection. In this review, the authors attempt to shed light on these important associations of issues that are not yet fully elucidated.
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spelling doaj.art-7243477f52794cb6844dea3c889e49172023-11-20T10:40:39ZengBMCRespiratory Research1465-993X2023-11-0124111010.1186/s12931-023-02593-1The impact of SARS-CoV-2 infection in patients with cystic fibrosis undergoing CFTR channel modulators treatment: a literature reviewAntonio Vitiello0Michela Sabbatucci1Andrea Silenzi2Annalisa Capuano3Francesco Rossi4Andrea Zovi5Francesco Blasi6Giovanni Rezza7Directorate General for Health Prevention, Ministry of HealthDirectorate General for Health Prevention, Ministry of HealthDirectorate General for Health Prevention, Ministry of HealthSection of Pharmacology “L. Donatelli”, Department of Experimental Medicine, Campania Regional Centre for Pharmacovigilance and Pharmacoepidemiology, University of Campania “Luigi Vanvitelli”Section of Pharmacology “L. Donatelli”, Department of Experimental Medicine, Campania Regional Centre for Pharmacovigilance and Pharmacoepidemiology, University of Campania “Luigi Vanvitelli”Directorate General for Hygiene, Food Safety and Nutrition, Ministry of HealthDepartment of Pathophysiology and Transplantation, University of MilanDirectorate General for Health Prevention, Ministry of HealthAbstract Several risk factors for Coronavirus-2019 (COVID-19) disease have been highlighted in clinical evidence. Among the various risk factors are advanced age, metabolic illness such as diabetes, heart disease, and diseases of the respiratory system. Cystic Fibrosis (CF) is a rare disease with autosomal recessive transmission, characterised by a lack of synthesis of the CFTR channel protein, and multi-organ clinical symptoms mainly affecting the respiratory tract with recurrent pulmonary exacerbations. In view of the pathophysiological mechanisms, CF disease should be in theory considered a risk factor for SARS-CoV2 or severe COVID-19. However, recent clinical evidence seems to point in the opposite direction, suggesting that CF could be a protective factor against severe COVID-19. Possibly, the lack of presence or function of the CFTR channel protein could be linked to the expression of the membrane glycoprotein ACE-2, a key enzyme for the endocellular penetration of SARS-CoV-2 and related to the pathophysiology of COVID-19 disease. Furthermore, CFTR channel modulating agents could indirectly influence the expression of ACE-2, playing an important role in restoring the proper functioning of mucociliary clearance and the pulmonary microbiome in the host response to SARS-CoV-2 infection. In this review, the authors attempt to shed light on these important associations of issues that are not yet fully elucidated.https://doi.org/10.1186/s12931-023-02593-1Cystic fibrosisCOVID-19CFTR modulatorsACE-2SARS-CoV-2Angiotensins
spellingShingle Antonio Vitiello
Michela Sabbatucci
Andrea Silenzi
Annalisa Capuano
Francesco Rossi
Andrea Zovi
Francesco Blasi
Giovanni Rezza
The impact of SARS-CoV-2 infection in patients with cystic fibrosis undergoing CFTR channel modulators treatment: a literature review
Respiratory Research
Cystic fibrosis
COVID-19
CFTR modulators
ACE-2
SARS-CoV-2
Angiotensins
title The impact of SARS-CoV-2 infection in patients with cystic fibrosis undergoing CFTR channel modulators treatment: a literature review
title_full The impact of SARS-CoV-2 infection in patients with cystic fibrosis undergoing CFTR channel modulators treatment: a literature review
title_fullStr The impact of SARS-CoV-2 infection in patients with cystic fibrosis undergoing CFTR channel modulators treatment: a literature review
title_full_unstemmed The impact of SARS-CoV-2 infection in patients with cystic fibrosis undergoing CFTR channel modulators treatment: a literature review
title_short The impact of SARS-CoV-2 infection in patients with cystic fibrosis undergoing CFTR channel modulators treatment: a literature review
title_sort impact of sars cov 2 infection in patients with cystic fibrosis undergoing cftr channel modulators treatment a literature review
topic Cystic fibrosis
COVID-19
CFTR modulators
ACE-2
SARS-CoV-2
Angiotensins
url https://doi.org/10.1186/s12931-023-02593-1
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