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J Cyst Fibros. 2018 Jan;17(1):83-88. doi: 10.1016/j.jcf.2017.06.002. Epub 2017 Jun 24.

Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor.

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Departments of Medicine and Pediatrics, Medical University of South Carolina, 96 Jonathan Lucas St, Room 812-CSB, MSC 630, Charleston, SC 29425, USA. Electronic address:
University of Queensland, Level 7, Centre for Child Health Research, Graham St, South Brisbane, Queensland 4101, Australia; Lady Cilento Children's Hospital, 501 Stanley St, South Brisbane 4101, Australia. Electronic address:
Division of Respirology, Keenan Research Centre of Li Ka Shing Knowledge Institute, Department of Medicine, St. Michael's Hospital, University of Toronto, 1 King's College Circle, 6263 Medical Sciences Building, Toronto, ON M5S 1A8, Canada. Electronic address:
Johnson & Johnson Medical Devices, 325 Paramount Dr, Raynham, MA 02767, USA. Electronic address:
Vertex Pharmaceuticals Incorporated, 50 Northern Avenue, Boston, MA 02210, USA. Electronic address:
Vertex Pharmaceuticals (Europe) Limited, 86-88 Jubilee Avenue, Milton Park, Abingdon, Oxfordshire OX14 4RW, UK. Electronic address:
National Heart and Lung Institute, Imperial College, London, UK; Department of Pediatric Respiratory Medicine, Royal Brompton and Harefield National Health Service Foundation Trust, Sydney Street, London SW3 6NP, UK. Electronic address:
Department of Pediatrics, University of Colorado School of Medicine, 13123 E 16th Ave, Aurora, CO 80045, USA. Electronic address:



Pulmonary exacerbations (PEx) are associated with acute loss of lung function that is often not recovered after treatment. We investigated lung function recovery following PEx for ivacaftor- and placebo-treated subjects.


Short- and long-term pulmonary function recovery data after PEx were summarized from a placebo-controlled trial in 161 cystic fibrosis patients≥12years old with the G551D-CFTR mutation (NCT00909532). Short-term recovery was measured 2 to 8weeks after treatment, and long-term recovery was determined at the end-of-study, both compared with baseline measured just prior to the PEx.


Fewer patients receiving ivacaftor experienced a PEx than patients receiving placebo (33.7% vs. 56.4%; P=0.004) and had a lower adjusted incidence rate of PEx (0.589 vs. 1.382; P<0.001). The proportion of PEx followed by full short-term recovery of percent predicted forced expiratory volume in 1s was similar (ivacaftor vs. placebo, 57.1% vs. 53.7), as was the proportion of patients having long-term recovery (46.4% vs. 47.7%).


Ivacaftor treatment reduces the frequency of PEx but does not improve on the rate of complete lung function recovery after PEx when compared with placebo.


Cystic fibrosis; Cystic fibrosis transmembrane conductance regulator; Ivacaftor; Pulmonary exacerbations; Pulmonary function

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