Improvement in Bronchiectasis on CT Imaging in a Pediatric Patient with Cystic Fibrosis on Ivacaftor Therapy

sis 11 years earlier. Her body mass index increased from 16.2 to 21.2 after 2 years of therapy, while her spirometry showed improvement in forced vital capacity from 2.02 liters (95% predicted) to 3.89 liters (118% predicted) and forced expiratory volume in 1 s from 1.57 liters (82% predicted) to 3.06 liters (105% predicted). Respiratory cultures continued to isolate methicillin-resistant Staphylococcus aureus . The year prior to starting ivacaftor therapy, the patient experienced two pulmonary exacerbations requiring oral antibiotics as an outpatient with no further antibiotic therapy required on ivacaftor therapy. Moreover, CT imaging of the chest (fig. 1) was also a useful tool to appraise response to ivacaftor. We believe the improvement in the lung disease radiographically was possible because the patient had cylindrical bronchiectasis (considered to be early-stage disease) before the development of saccular or varicose bronchiectasis, which is believed to be irreversible.