Abstract
Cystic Fibrosis (CF) is a recessive genetic disease due to mutations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene encoding the CFTR chloride channel. The ion transport abnormalities related to CFTR mutation generate a dehydrated airway surface liquid (ASL) layer, which is responsible for an altered mucociliary clearance, favors infections and persistent inflammation that lead to progressive lung destruction and respiratory failure. The inflammatory response is normally followed by an active resolution phase to return to tissue homeostasis, which involves specialized pro-resolving mediators (SPMs). SPMs promote resolution of inflammation, clearance of microbes, tissue regeneration and reduce pain, but do not evoke unwanted immunosuppression. The airways of CF patients showed a decreased production of SPMs even in the absence of pathogens. SPMs levels in the airway correlated with CF patients’ lung function. The prognosis for CF has greatly improved but there remains a critical need for more effective treatments that prevent excessive inflammation, lung damage, and declining pulmonary function for all CF patients. This review aims to highlight the recent understanding of CF airway inflammation and the possible impact of SPMs on functions that are altered in CF airways.
Highlights
Cystic Fibrosis (CF) is a multisystemic disorder with the lung disease being the main cause of morbidity and mortality
This review aims to highlight the recent understanding of CF airway inflammation and the possible impact of specialized pro-resolving mediators (SPMs) on functions that are altered in CF airways
The combination of advanced knowledge in the field of the regulation of innate immunity and the current description of CF airway disease revealed novel cellular and molecular mechanisms involved in the abnormal resolution of inflammation in CF
Summary
CF is a multisystemic disorder with the lung disease being the main cause of morbidity and mortality. It is commonly acknowledged that the reduced ASL height in CF, is responsible for an altered mucociliary clearance which favors infections and persistent inflammation, leading to progressive lung destruction and respiratory failure (Davis, 2006). CF airway inflammation is excessive and ineffective against pathogens, can occur very early in the development of the disease and in some cases without any sign of infection (Balough et al, 1995; Khan et al, 1995; Armstrong et al, 1997; Balázs and Mall, 2019). The inflammatory response is normally followed by an active resolution phase to return to tissue homeostasis in which among other mediators (cytokines, chemokines, immune cells), bioactive lipids might play a crucial role.
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