An update on targeting airway inflammation in cystic fibrosis

Abstract

Introduction: For many years the major cause of morbidity and mortality in people with cystic fibrosis (PWCF) was lung disease, characterized by progressive airway inflammation, bacterial colonization and premature death. With the advent of highly effective modulator therapies (HEMT) many of the parameters predicting premature death have been beneficially altered with resultant predicted improvement in survival. It is unknown how much residual airway inflammation will persist in PWCF on HEMT, whether bacterial colonization will be eradicated and whether the beneficial effects decrease over times. In addition, a significant number of PWCF cannot avail of HEMT.

Areas covered: We discuss pathogenesis of airways disease in CF, airway inflammation and bacterial colonization in PWCF on and off HEMT, and whether new anti-inflammatory strategies are required to decrease residual inflammation and improve bacterial eradication. We discuss the potential therapeutic options for those PWCF for whom HEMT are not an option.

Expert opinion: PWCF on HEMT may expect prolonged survival but in many, particularly those in whom HEMT therapy was instituted after the onset of structural lung disease there will be persistent inflammation requiring further therapy.

Keywords: Cystic fibrosis; anti-inflammatory therapies; clinical trials; highly effective modulator therapy, inflammation; neutrophils.