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CFTR primarily functions as a chloride channel that transports ions across the apical membrane of epithelial cells but has other functions, including bicarbonate secretion and inhibition of sodium transport. Defective CFTR disrupts these functions, causing viscous and dehydrated mucus to accumulate, compromising the airway lumen and contributing to obstructive pulmonary disease. The combination of CFTR dysfunction, mucus obstruction, and infection drive an exaggerated and dysfunctional inflammatory response, which contributes to irreversible airway destruction and fibrosis.<\/jats:p><jats:p>CFTR modulators, an exciting new class of drugs, increase the expression and\/or function of CFTR variant protein and improve multiple clinical endpoints, such as lung function, pulmonary exacerbation rates, and nutritional status. However, these genotype\u2010specific drugs are not universally available, the clinical response is variable, and lung function still declines over time when bronchiectasis is established. Consequently, even in the age of CFTR modulators, we must target other important aspects of the CF airway disease, such as inflammation and mucociliary clearance. This review highlights the mechanisms of inflammation and mucus accumulation in the CF lung and discusses anti\u2010inflammatory and mucociliary clearance agents that are currently in development focusing on compounds for which clinical trial data have recently become available.<\/jats:p>","DOI":"10.1002\/ppul.24364","type":"journal-article","created":{"date-parts":[[2019,11,12]],"date-time":"2019-11-12T19:06:51Z","timestamp":1573585611000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Anti\u2010inflammatories and mucociliary clearance therapies in the age of CFTR modulators"],"prefix":"10.1002","volume":"54","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4970-8889","authenticated-orcid":false,"given":"Lucy","family":"Perrem","sequence":"first","affiliation":[{"name":"Division of Respiratory Medicine, The Department of Paediatrics The Hospital for Sick Children Toronto Ontario Canada"},{"name":"University of Toronto Toronto Ontario Canada"},{"name":"Translational Medicine Program Research Institute, Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4057-6592","authenticated-orcid":false,"given":"Felix","family":"Ratjen","sequence":"additional","affiliation":[{"name":"Division of Respiratory Medicine, The Department of Paediatrics The Hospital for Sick Children Toronto Ontario Canada"},{"name":"University of Toronto Toronto Ontario Canada"},{"name":"Translational Medicine Program Research Institute, Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,11,12]]},"reference":[{"key":"e_1_2_13_1_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrdp.2015.10"},{"key":"e_1_2_13_1_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1300109"},{"key":"e_1_2_13_1_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1105185"},{"key":"e_1_2_13_1_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2014.09.005"},{"key":"e_1_2_13_1_6_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201404-0703OC"},{"key":"e_1_2_13_1_7_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201609-1954OC"},{"key":"e_1_2_13_1_8_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201503-0578OC"},{"key":"e_1_2_13_1_9_1","doi-asserted-by":"crossref","unstructured":"A current view on inflammation. 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