Lung Microbiome in Cystic Fibrosis

The defective mucociliary clearance due to CFTR malfunctioning causes predisposition to the colonization of pathogens responsible for the recurrent inflammation and rapid deterioration of lung function in patients with cystic fibrosis (CF). This has also a profound effect on the lung microbiome composition, causing a progressive reduction in its diversity, which has become a common characteristic of patients affected by CF. Although we know that the lung microbiome plays an essential role in maintaining lung physiology, our comprehension of how the microbial components interact with each other and the lung, as well as how these interactions change during the disease's course, is still at an early stage. Many challenges exist and many questions still to be answered, but there is no doubt that manipulation of the lung microbiome could help to develop better therapies for people affected by CF.

Automated summary

The defective mucociliary clearance in CF patients due to CFTR malfunctioning predisposes them to colonization by pathogens, leading to recurrent inflammation and rapid deterioration of lung function, impacting the lung microbiome composition. Understanding of microbial interactions in the lung and how they change during disease progression is still in its early stages, but manipulating the lung microbiome could offer promising prospects for improving therapies for CF patients.