The Impact of Air Pollution on the Course of Cystic Fibrosis: A Review

Abstract

Cystic fibrosis (CF) is a lethal and widespread autosomal recessive disorder affecting over 80,000 people worldwide. It is caused by mutations of the CFTR gene, which encodes an epithelial anion channel. CF is characterized by a great phenotypic variability which is currently not fully understood. Although CF is genetically determined, the course of the disease might also depend on multiple other factors. Air pollution, whose effects on health and contribution to respiratory diseases are well established, is one environmental factor suspected to modulate the disease severity and influence the lung phenotype of CF patients. This is of particular interest as pulmonary failure is the primary cause of death in CF. The present review discusses current knowledge on the impact of air pollution on CF pathogenesis and aims to explore the underlying cellular and biological mechanisms involved in these effects.

ERS International Congress 2022: highlights from the Respiratory Infections Assembly

“…In cystic fibrosis, air pollution is associated with increased frequency of exacerbations and risk of initial acquisition of P. aeruginosa and S. aureus , and decreased lung function [ 69 ]. Using the Scnn1b -transgenic (Tg) mouse model that overexpresses the epithelial Na + channel producing a cystic fibrosis-like phenotype, Marion Blayac (Créteil, France) simulated atmospheres representative of an urban city and a highly polluted megacity, taking into account seasonal variation to examine the effects of air pollution in cystic fibrosis.…”

Social-environmental phenotypes of rapid cystic fibrosis lung disease progression in adolescents and young adults living in the United States

“…Furthermore, the effects of social and environmental exposures on CF lung function decline have primarily been studied one exposure domain at a time. Examples of two separate but frequently studied exposures are air pollution ( Blayac et al, 2022 ) and Medicaid insurance use ( Schechter et al, 2001 ). While the former has been linked primarily to pulmonary exacerbation onset ( Goeminne et al, 2013 ; Goss et al, 2004 ), which is largely driven by acute drops in lung function, the latter has often corresponded to prolonged drops in lung function ( Schechter and Margolis, 1998 ).…”