Ivacaftor decreases monocyte sensitivity to interferon-γ in people with cystic fibrosis. Issue 2 (19th April 2020)

• Record Type: Journal Article
• Authors: Hisert, Katherine B.; Birkland, Timothy P.; Schoenfelt, Kelly Q.; Long, Matthew E.; Grogan, Brenda; Carter, Suzanne; Liles, W. Conrad; McKone, Edward F.; Becker, Lev; Manicone, Anne M.
• Is Part Of: ERJ open research. Volume 6:Issue 2(2020)
• Publication Date: 2020-04-19
• Subjects: Respiratory organs -- Diseases -- Periodicals; Respiration -- Periodicals; Respiration; Respiratory organs -- Diseases; Respiratory organs -- Diseases -- Treatment; Respiratory Tract Diseases; Electronic journals; Fulltext; Internet Resources; Periodicals; Periodical; 616.2005
• DOI: 10.1183/23120541.00318-2019
• Languages: English
• ISSNs: 2312-0541

Abstract

Management of cystic fibrosis has been revolutionised by the introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulators. These compounds treat the underlying molecular basis of the disease by increasing activity of defective CFTR channels, which improves many clinical parameters and enhances patient quality of life [1]. Next-generation modulators, also known as triple combination therapy, promise to be highly efficacious in up to 90% of patients [2] and will likely dramatically change the landscape of cystic fibrosis disease. Studies examining individuals before and after initiation of CFTR modulators have revealed novel functions of CFTR and shown that CFTR modulators do not reverse all disease manifestations [3–5]. Thus, knowledge of the post-modulator cystic fibrosis disease state is crucial for understanding what continued therapies will be needed for people with cystic fibrosis and what new challenges may arise. This study demonstrates that initiation of the CFTR modulator ivacaftor in people with cystic fibrosis and susceptible CFTR mutations causes an acute reduction in blood monocyte sensitivity to the key proinflammatory cytokine IFN-γ http://bit.ly/2TeI6LG