IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES Ivacaftor Imaging Response in Cystic Fibrosis Siobhan Hoare1, Sinead McEvoy1, Colin J. McCarthy1, Aoife Kilcoyne1, Darragh Brady1, Brian Gibney1, Charles G. Gallagher2, Edward F. McKone2, and Jonathan D. Dodd1 1
Department of Radiology and 2National Referral Centre for Adult Cystic Fibrosis, St. Vincent’s University Hospital, Dublin, Ireland
Figure 1.
A 27-year-old Irish adult with cystic fibrosis (CF; DF508/G551D genotype) was commenced on ivacaftor in January 2011. Ivacaftor addresses the underlying cause of CF in individuals with the G551D genotype by increasing chloride transport through cell surface CF transmembrane conductance regulator. Before this, he suffered two to three respiratory exacerbations per year, none requiring hospital admission. Sputum was positive for Staphylococcus aureus and Pseudomonas aeruginosa. After 2 years of treatment, the patient’s FEV1 increased from 79% predicted to 95% predicted, and he was completely free of respiratory exacerbations during this time. A previous chest computed tomography scan (CT) in 2011 showed mild diffuse bilateral upper lobe bronchiectasis (Figure 1A, arrowhead), mild diffuse airway wall thickening (straight arrows) and diffuse mucus plugging (curved arrows). A follow-up chest CT in 2013 showed stabilization of bronchiectasis (Figure 1B, arrowhead), marked improvement in airway wall thickening (straight arrows), and extensive clearing of mucus plugging (curved arrows). The stabilization of bronchiectasis and reduction in airway thickening and mucus plugging demonstrated by CT in this patient correlate with his improvement in lung function and are likely a result of chronic therapy with ivacaftor. High-resolution CT imaging may be a useful tool to gauge response to therapy with ivacaftor for patients with CF with the G551D mutation. n Author disclosures are available with the text of this article at www.atsjournals.org.
Am J Respir Crit Care Med Vol 189, Iss 4, p 484, Feb 15, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201308-1433IM Internet address: www.atsjournals.org
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American Journal of Respiratory and Critical Care Medicine Volume 189 Number 4 | February 15 2014
Department of Radiology and 2National Referral Centre for Adult Cystic Fibrosis, St. Vincent’s University Hospital, Dublin, Ireland
Figure 1.
A 27-year-old Irish adult with cystic fibrosis (CF; DF508/G551D genotype) was commenced on ivacaftor in January 2011. Ivacaftor addresses the underlying cause of CF in individuals with the G551D genotype by increasing chloride transport through cell surface CF transmembrane conductance regulator. Before this, he suffered two to three respiratory exacerbations per year, none requiring hospital admission. Sputum was positive for Staphylococcus aureus and Pseudomonas aeruginosa. After 2 years of treatment, the patient’s FEV1 increased from 79% predicted to 95% predicted, and he was completely free of respiratory exacerbations during this time. A previous chest computed tomography scan (CT) in 2011 showed mild diffuse bilateral upper lobe bronchiectasis (Figure 1A, arrowhead), mild diffuse airway wall thickening (straight arrows) and diffuse mucus plugging (curved arrows). A follow-up chest CT in 2013 showed stabilization of bronchiectasis (Figure 1B, arrowhead), marked improvement in airway wall thickening (straight arrows), and extensive clearing of mucus plugging (curved arrows). The stabilization of bronchiectasis and reduction in airway thickening and mucus plugging demonstrated by CT in this patient correlate with his improvement in lung function and are likely a result of chronic therapy with ivacaftor. High-resolution CT imaging may be a useful tool to gauge response to therapy with ivacaftor for patients with CF with the G551D mutation. n Author disclosures are available with the text of this article at www.atsjournals.org.
Am J Respir Crit Care Med Vol 189, Iss 4, p 484, Feb 15, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201308-1433IM Internet address: www.atsjournals.org
484
American Journal of Respiratory and Critical Care Medicine Volume 189 Number 4 | February 15 2014
