Immunomodulation in Cystic Fibrosis: Why and How?

作者：Vincent D. Giacalone, Brian Dobosh, Amit Gaggar, Rabindra Tirouvanziam, Camilla Margaroli · 发表于：International Journal of Molecular Sciences · 年份：2020 · DOI：10.3390/ijms21093331 · 被引用次数：25 · 研究领域：Cystic Fibrosis Research Advances、Neonatal Respiratory Health Research、Inhalation and Respiratory Drug Delivery

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.