Potential systemic effects of acquired CFTR dysfunction in COPD

Respir Med. 2023 Dec 15:107499. doi: 10.1016/j.rmed.2023.107499. Online ahead of print.ABSTRACTChronic obstructive pulmonary disease (COPD) is characterized by airflow limitation, respiratory symptoms, inflammation of the airways, and systemic manifestations of the disease. Genetic susceptibility and environmental factors are important in the development of the disease, particularly exposure to cigarette smoke which is the most notable risk factor. Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene are the cause of cystic fibrosis (CF), which shares several pathophysiological pulmonary features with COPD, including airway obstruction, chronic airway inflammation and bacterial colonization; in addition, both diseases also present systemic defects leading to comorbidities such as pancreatic, gastrointestinal, and bone-related diseases. In patients with COPD, systemic CFTR dysfunction can be acquired by cigarette smoking, inflammation, and infection. This dysfunction is, on average, about half of that found in CF. Herein we review the literature focusing on acquired CFTR dysfunction and the potential role in the pathogenesis of comorbidities associated with COPD and chronic bronchitis.PMID:38104786 | DOI:10.1016/j.rmed.2023.107499

Source: Respiratory Care - December 17, 2023 Category: Respiratory Medicine Authors: Marc Miravitlles Gerard J Criner Marcus A Mall Steven M Rowe Claus F Vogelmeier Bettina Hederer Matthias Schoenberger Pablo Altman Source Type: research