TY - JOUR T1 - Ivacaftor decreases monocyte sensitivity to interferon-γ in people with cystic fibrosis JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00318-2019 VL - 6 IS - 2 SP - 00318-2019 AU - Katherine B. Hisert AU - Timothy P. Birkland AU - Kelly Q. Schoenfelt AU - Matthew E. Long AU - Brenda Grogan AU - Suzanne Carter AU - W. Conrad Liles AU - Edward F. McKone AU - Lev Becker AU - Anne M. Manicone Y1 - 2020/04/01 UR - http://openres.ersjournals.com/content/6/2/00318-2019.abstract N2 - 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/2TeI6LGWe thank Pradeep Singh (University of Washington, Seattle, WA, USA) for inviting us to join the research team in Dublin, Ireland; we also thank Michael Welsh (University of Iowa, Iowa City, IA, USA), David Stoltz (University of Iowa), Jan Launspach (University of Iowa), Mavra Nasir (Dartmouth University, Hanover, NH, USA), Anina Ratjen (University of Washington), Robert Gray (University of Edinburgh, Edinburgh, UK) and Gareth Hardisty (University of Edinburgh) for collegial interactions and strategic support during specimen collection and initial specimen processing in Dublin. ER -