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Immunomodulatory Cell Therapy to Target Cystic Fibrosis Inflammation.

ABSTRACT

Cystic fibrosis (CF) is associated with exaggerated and prolonged inflammation in the lungs, which contributes to lung injury, airway mucus obstruction, bronchiectasis and loss of lung function. This hyper-inflammatory phenotype appears to be caused by an imbalance between the pro- and anti-inflammatory regulatory pathways, with heightened pro-inflammatory stimuli, a decreased counter-regulatory response, and reduced effectiveness of immune cell function and inflammatory resolution. Thus, therapies that can target this inflammatory environment would have a major impact in preventing the progression of lung disease. Due to the complex phenotype of CF inflammation, current anti-inflammatory regimens have proven to be inadequate for the targeting of these multiple dysregulated pathways and effects. Several approaches utilizing cell therapies have shown potential therapeutic benefit for the treatment of CF inflammation. This review provides an overview of the immune dysfunctions in CF and current therapeutic regimens and explores the field of cell therapy as a treatment for CF inflammation, and focuses on the various cell types utilized, their immunomodulatory functions, and the current approaches to mitigate the inflammatory response and reduce the long-term damage for CF patients.