TABLE 1

Results of forced expiratory volume in 1 s (FEV1) and cystic fibrosis (CF) computed tomography (CT) score

Baseline CT (T0)Short-term follow-up (T1)Long-term follow-up (T2)T1 versus T0T2 versus T0T2 versus T1
Pseudomedian (95% CI)p-valuePseudomedian (95% CI)p-valuePseudomedian (95% CI)p-value
Patients n22141614168
Time from introduction  of ivacaftor months−4.1 (–12.4–0.8)8 (6.1–11.7)33.1 (30.8–36.7)
FEV1 % pred39.5 (31.5–77)54 (34.3–81.8)48.5 (35.0–81.8)9.0 (3.0–15.5)0.0075.5 (1.0–11.0)0.0361.7 (–10.5–7.0)1
CF-CT score#33.4 (22.3–41.1)29.8 (22.5–36.6)32.7 (17.8–36.8)–5.1 (–9.6– –0.5)0.017–3.1 (–5.8– –1.1)0.011–0.5 (–4.4–4.7)0.945
 Bronchiectasis33.7 (13.4–51.3)34.5 (23.6–55.7)39.9 (18.2–52.6)4.6 (–0.9–9.5)0.1335.0 (1.5–8.1)0.0252.3 (–2.0–7.3)0.313
 Peribronchial   thickening43.5 (36.3–55.4)43.5 (27.0–48.1)40.8 (27.8–48.1)–9.3 (–17.6– –0.90)0.042–5.8 (–17.3– –3.1)0.002–1.1 (–8.3–6.6)0.833
 Mucus plugging43.1 (24.3–56.7)29.2 (19.4–35.4)23.6 (12.5–29.2)–18.1 (–30.5– –8.4)0.001–19.4 (–32.0– –4.2)0.011–7.3 (–16.6–9.2)0.313
 Parenchymal   abnormalities7.4 (3.9–10.7)8.2 (3.7–10.7)7.1 (3.3–7.4)–1.9 (–3.7–0.4)0.083–1.8 (–2.8–0.2)0.1191.7 (–1.8–3.7)0.787
  • Data are presented as median (interquartile range), unless otherwise stated. Comparison is assessed using paired Wilcoxon test and results. #: percentage of maximum possible scores.