Tracheal Diverticula in People with Cystic Fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: An Italian Multicenter Retrospective Study

<b>Background/Objectives</b>: Cystic Fibrosis (CF) is an autosomal recessive genetic disorder caused by variants in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Recently, a targeted therapy for CF has been developed, represented by the CFTR modulators that enhance or restore the function of the CFTR protein. The most recent is the combination of three modulators, Elexacaftor, Tezacaftor, and Ivacaftor (ETI). This study describes the presentation, management, and follow-up of tracheal diverticulum (TD) in pwCF receiving ETI therapy. <b>Methods</b>: This retrospective study included people with CF (pwCF) on ETI treatment and followed up in two CF Italian centers who developed an asymptomatic TD, diagnosed incidentally at chest CT scan. <b>Results</b>: Among 268 pwCF receiving ETI, three (1.19%) were diagnosed with TD identified after chest CT and were included in this study. Endoscopic confirmation was obtained in one patient. All patients were on inhaled colistimethate, two of them for chronic <i>Pseudomonas aeruginosa</i> colonization, and one undergoing eradication therapy. <b>Conclusions</b>: TD may be identified in chest CT obtained in pwCF in treatment with ETI. Further studies and a longer follow up are needed to confirm these findings.

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Journal
Journal of Clinical Medicine
Published
2025-03-28
DOI
https://doi.org/10.3390/jcm14072320
Primary Topic
Cystic Fibrosis Research Advances
Type
article
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article

Tracheal Diverticula in People with Cystic Fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: An Italian Multicenter Retrospective Study

Roberto Baggi, Stefano Avenali, Marco Di Maurizio, Angela Sepe et al.
Journal of Clinical Medicine
Cystic Fibrosis Research Advances
article

Tracheal Diverticula in People with Cystic Fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: An Italian Multicenter Retrospective Study

Roberto Baggi, Stefano Avenali, Marco Di Maurizio, Angela Sepe, Antonella Tosco, Cristina Fevola, Vito Terlizzi, Laura Venditto, Chiara Cimbalo, Alice Castaldo
article en

Abstract

<b>Background/Objectives</b>: Cystic Fibrosis (CF) is an autosomal recessive genetic disorder caused by variants in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Recently, a targeted therapy for CF has been developed, represented by the CFTR modulators that enhance or restore the function of the CFTR protein. The most recent is the combination of three modulators, Elexacaftor, Tezacaftor, and Ivacaftor (ETI). This study describes the presentation, management, and follow-up of tracheal diverticulum (TD) in pwCF receiving ETI therapy. <b>Methods</b>: This retrospective study included people with CF (pwCF) on ETI treatment and followed up in two CF Italian centers who developed an asymptomatic TD, diagnosed incidentally at chest CT scan. <b>Results</b>: Among 268 pwCF receiving ETI, three (1.19%) were diagnosed with TD identified after chest CT and were included in this study. Endoscopic confirmation was obtained in one patient. All patients were on inhaled colistimethate, two of them for chronic <i>Pseudomonas aeruginosa</i> colonization, and one undergoing eradication therapy. <b>Conclusions</b>: TD may be identified in chest CT obtained in pwCF in treatment with ETI. Further studies and a longer follow up are needed to confirm these findings.

Journal of Clinical MedicineVol. 14(7)
Regione Campania (IT), Azienda Ospedaliera Universitaria Integrata Verona (IT), Meyer Children's Hospital (IT), Federico II University Hospital (IT), Istituti di Ricovero e Cura a Carattere Scientifico (IT)
Good health and well-being
Openalex Percentile: Top 91%
Cystic Fibrosis Research Advances
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