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Potential systemic effects of acquired CFTR dysfunction in COPD.

Marc Miravitlles¹, Gerard J Criner², Marcus A Mall³

¹Pneumology Department Hospital Universitari Vall d'Hebron, Vall d'Hebron Institut de Recerca (VHIR), Vall d'Hebron Hospital Campus, Barcelona, Spain.

Respiratory Medicine

|December 17, 2023

Summary

This summary is machine-generated.

Cigarette smoking and inflammation can cause cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction in chronic obstructive pulmonary disease (COPD) patients. This acquired dysfunction may contribute to COPD comorbidities.

Keywords:

CFTR dysfunction CFTR potentiator COPD Chronic bronchitis Cigarette smoke Icenticaftor Multi-organ disease

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Area of Science:

Pulmonary Medicine
Genetics
Respiratory Diseases

Background:

Chronic obstructive pulmonary disease (COPD) involves airflow limitation, airway inflammation, and systemic effects.
Cystic fibrosis (CF), caused by CFTR gene mutations, shares airway obstruction and inflammation features with COPD.
Both CF and COPD can lead to systemic comorbidities like pancreatic, gastrointestinal, and bone diseases.

Purpose of the Study:

To review literature on acquired CFTR dysfunction in COPD.
To explore the role of acquired CFTR dysfunction in COPD pathogenesis and comorbidities.

Main Methods:

Literature review of studies on CFTR dysfunction in COPD.
Analysis of shared pathophysiological features between CF and COPD.
Examination of acquired CFTR dysfunction mechanisms in COPD.

Main Results:

COPD patients can acquire systemic CFTR dysfunction due to smoking, inflammation, and infection.
Acquired CFTR dysfunction in COPD is approximately half as severe as in CF.
This dysfunction is implicated in the pathogenesis of COPD-related comorbidities.

Conclusions:

Acquired CFTR dysfunction is a significant factor in COPD pathogenesis.
Targeting CFTR dysfunction may offer therapeutic potential for COPD comorbidities.
Further research is needed to fully elucidate the role of CFTR in COPD.