Immunomodulation in Cystic Fibrosis: Why and How?

• Published in: International journal of molecular sciences Vol. 21; no. 9; p. 3331
• Main Authors: Giacalone, Vincent D, Dobosh, Brian S, Gaggar, Amit, Tirouvanziam, Rabindra, Margaroli, Camilla
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 08.05.2020; MDPI
• Subjects: Animals; Apoptosis; Asthma; B-Lymphocytes - drug effects; B-Lymphocytes - immunology; B-Lymphocytes - pathology; Bacterial infections; Conductance; Cystic fibrosis; Cystic Fibrosis - enzymology; Cystic Fibrosis - immunology; Cystic Fibrosis - therapy; Cystic Fibrosis Transmembrane Conductance Regulator - metabolism; Granulocytes; Granulocytes - drug effects; Granulocytes - immunology; Granulocytes - pathology; Humans; Immune response; Immune system; Immunomodulation; Immunomodulation - drug effects; immunotherapy; Immunotherapy - methods; Infections; Inflammation; Inflammation - drug therapy; Inflammation - immunology; Inflammation - metabolism; Inflammation - therapy; Lavage; Lung - immunology; Lung - pathology; lung disease; Lung diseases; Lungs; Macrophages - drug effects; Macrophages - immunology; Mast Cells - drug effects; Mast Cells - immunology; Mast Cells - pathology; Matrix Metalloproteinase Inhibitors - pharmacology; Mutation; Neutrophils; Neutrophils - drug effects; Neutrophils - enzymology; Neutrophils - immunology; Neutrophils - metabolism; Nucleic Acids - therapeutic use; Pathogens; Pathology; proteases; Review; Side effects; T-Lymphocytes - drug effects; T-Lymphocytes - immunology; T-Lymphocytes - pathology; Viral infections; lung disease; inflammation; cystic fibrosis; immunotherapy; proteases
• ISSN: 1422-0067; 1661-6596; 1422-0067
• DOI: 10.3390/ijms21093331

Cystic fibrosis (CF) lung disease is characterized by unconventional mechanisms of inflammation, implicating a chronic immune response dominated by innate immune cells. Historically, therapeutic development has focused on the mutated cystic fibrosis transmembrane conductance regulator (CFTR), leading to the discovery of small molecules aiming at modulating and potentiating the presence and activity of CFTR at the plasma membrane. However, treatment burden sustained by CF patients, side effects of current medications, and recent advances in other therapeutic areas have highlighted the need to develop novel disease targeting of the inflammatory component driving CF lung damage. Furthermore, current issues with standard treatment emphasize the need for directed lung therapies that could minimize systemic side effects. Here, we summarize current treatment used to target immune cells in the lungs, and highlight potential benefits and caveats of novel therapeutic strategies.