What is it about?
Cystic fibrosis (CF) lung disease is characterized by chronic infection with and sustained inflammation. The lack of effective anti-inflammatory therapies for people with CF (PWCF) represents a significant challenge. Here we show that immune cells called neutrophils alter their metabolism in CF in response to inflammation and infection. This shift results in further production of inflammatory proteins by these cells, fueling a vicious cycle of inflammation in the airway. In this study we identify key targets for therapies directed at this pathway, and demonstrate a protective effect of one such therapy, MCC950, in an animal model of CF.
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Why is it important?
The neutrophil is the key immune cell involved in the pathogenesis of CF lung disease. Here we show that the metabolism of these cells is altered in response to inflammation, and that this signal can be detected in the blood as well as the lung. We also demonstrate for the first time that specific inhibition of the NLRP3 inflammasome in vivo decreases CF airway inflammation and reduces infection with Pseudomonas aeruginosa. This provides us with a new strategy to tackle inflammation and infection in people with CF.
Perspectives
This study was technically challenging but highly rewarding. It involved strong collaboration with international experts in the fields of pulmonology and immunology, and stands to fuel the development of new therapies for CF patients.
Oliver McElvaney
Royal College of Surgeons in Ireland
Read the Original
This page is a summary of: Specific Inhibition of the NLRP3 Inflammasome as an Antiinflammatory Strategy in Cystic Fibrosis, American Journal of Respiratory and Critical Care Medicine, December 2019, American Thoracic Society,
DOI: 10.1164/rccm.201905-1013oc.
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