IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES Improvement of Sinus Disease in Cystic Fibrosis with Ivacaftor Therapy Don Hayes, Jr., Karen S. McCoy, and Shahid I. Sheikh Department of Pediatrics, The Ohio State University College of Medicine, Nationwide Children’s Hospital, Columbus, Ohio
Figure 1. Coronal view of the maxillofacial computed tomography scan without contrast demonstrating (A) diffuse sinus opacification and (B) significant improvement in both sinus opacification and mucosal thickening with ivacaftor therapy.
A 19-year-old female with cystic fibrosis (DF508/G551D genotype) was started on ivacaftor therapy, which corrects the gating defect of the cystic fibrosis transmembrane conductance regulator (CFTR) channel associated with the G551D mutation. Before starting therapy, she experienced chronic sinus disease with nasal congestion, facial pressure, and associated headaches requiring functional endoscopic sinus surgery on two separate occasions earlier in adolescence. She was otherwise healthy, with spirometry showing FVC 3.04 L (87% predicted) and FEV1 2.39 L (77% predicted) and computed tomography (CT) scan of the chest showing mild cylindrical bronchiectasis in the upper lobes. With a history of allergic rhinitis, treatment that was started during early adolescence in 2005, including saline nasal wash, fluticasone propionate nasal spray, and montelukast sodium, was continued. After 1.5 years of ivacaftor treatment, her sweat chloride decreased from 124 and 113 mmol/L (at time of diagnosis in 1992) to 38 and 40 mmol/L, while her body mass index increased from 20.5 to 23.2 kg/m2. Follow-up spirometry showed improvement in FVC to 3.49 L (100% predicted) and FEV1 to 3.19 L (103% predicted). On ivacaftor therapy, respiratory cultures persisted to isolate mucoidal Pseudomonas aeruginosa and methicillin-resistant Staphylococcus aureus. She experienced no pulmonary exacerbations during this 1.5-year time period, and her sinus symptoms completely resolved, with maxillofacial CT showing significant improvement (Figure 1). Based on our experiences, maxillofacial CT is a useful tool to appraise response of sinus disease to ivacaftor for patients with CF with CFTR-G551D mutation. n Author disclosures are available with the text of this article at www.atsjournals.org.
Author Contributions: Conception and design: D.H., K.S.M., and S.I.S. Analysis and interpretation: D.H., K.S.M., and S.I.S. Drafting the manuscript: D.H. Am J Respir Crit Care Med Vol 190, Iss 4, p 468, Aug 15, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201403-0595IM Internet address: www.atsjournals.org
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American Journal of Respiratory and Critical Care Medicine Volume 190 Number 4 | August 15 2014
Figure 1. Coronal view of the maxillofacial computed tomography scan without contrast demonstrating (A) diffuse sinus opacification and (B) significant improvement in both sinus opacification and mucosal thickening with ivacaftor therapy.
A 19-year-old female with cystic fibrosis (DF508/G551D genotype) was started on ivacaftor therapy, which corrects the gating defect of the cystic fibrosis transmembrane conductance regulator (CFTR) channel associated with the G551D mutation. Before starting therapy, she experienced chronic sinus disease with nasal congestion, facial pressure, and associated headaches requiring functional endoscopic sinus surgery on two separate occasions earlier in adolescence. She was otherwise healthy, with spirometry showing FVC 3.04 L (87% predicted) and FEV1 2.39 L (77% predicted) and computed tomography (CT) scan of the chest showing mild cylindrical bronchiectasis in the upper lobes. With a history of allergic rhinitis, treatment that was started during early adolescence in 2005, including saline nasal wash, fluticasone propionate nasal spray, and montelukast sodium, was continued. After 1.5 years of ivacaftor treatment, her sweat chloride decreased from 124 and 113 mmol/L (at time of diagnosis in 1992) to 38 and 40 mmol/L, while her body mass index increased from 20.5 to 23.2 kg/m2. Follow-up spirometry showed improvement in FVC to 3.49 L (100% predicted) and FEV1 to 3.19 L (103% predicted). On ivacaftor therapy, respiratory cultures persisted to isolate mucoidal Pseudomonas aeruginosa and methicillin-resistant Staphylococcus aureus. She experienced no pulmonary exacerbations during this 1.5-year time period, and her sinus symptoms completely resolved, with maxillofacial CT showing significant improvement (Figure 1). Based on our experiences, maxillofacial CT is a useful tool to appraise response of sinus disease to ivacaftor for patients with CF with CFTR-G551D mutation. n Author disclosures are available with the text of this article at www.atsjournals.org.
Author Contributions: Conception and design: D.H., K.S.M., and S.I.S. Analysis and interpretation: D.H., K.S.M., and S.I.S. Drafting the manuscript: D.H. Am J Respir Crit Care Med Vol 190, Iss 4, p 468, Aug 15, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201403-0595IM Internet address: www.atsjournals.org
468
American Journal of Respiratory and Critical Care Medicine Volume 190 Number 4 | August 15 2014
