Patients n | 22 | 14 | 16 | 14 | | 16 | | 8 | |
Time from introduction of ivacaftor months | −4.1 (–12.4–0.8) | 8 (6.1–11.7) | 33.1 (30.8–36.7) | | | | | | |
FEV1 % pred | 39.5 (31.5–77) | 54 (34.3–81.8) | 48.5 (35.0–81.8) | 9.0 (3.0–15.5) | 0.007 | 5.5 (1.0–11.0) | 0.036 | 1.7 (–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 |
Bronchiectasis | 33.7 (13.4–51.3) | 34.5 (23.6–55.7) | 39.9 (18.2–52.6) | 4.6 (–0.9–9.5) | 0.133 | 5.0 (1.5–8.1) | 0.025 | 2.3 (–2.0–7.3) | 0.313 |
Peribronchial thickening | 43.5 (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 plugging | 43.1 (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 abnormalities | 7.4 (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) | 0.119 | 1.7 (–1.8–3.7) | 0.787 |