Switch-IPF cohort (n = 37) | Non-Switch-IPF cohort (n = 225) | Non-Switch-IPF Continued cases (n = 177) | Non-Switch-IPF Discontinued cases (n = 48) | Non-Switch IPF versus Switch-IPF p value | |
---|---|---|---|---|---|
Age, years | 70.0 [65.5–74.0] | 73.0 [68.0–77.0] | 72.0 [67.5–76.0] | 74.0 [68.0–79.0] | 0.0616 |
Sex, male/female | 31 (83.8%)/6 (16.2%) | 185 (82.2%)/40 (17.8%) | 147 (83.0%)/30 (17.0%) | 38 (79.2%)/10 (20.8%) | 1.000 |
cIPF/UIP/IPF | 31 (83.8%)/6 (16.2%) | 175 (77.8%)/50 (22.2%) | 134 (75.7%)/43 (24.3%) | 41 (85.4%)/7 (14.6%) | 0.5186 |
Diagnosis to antifibrotic therapy, months | 13.6 [1.7–31.5] | 15.4 [2.8–47.2] | 12.1 [2.6–44.1] | 31.5 [5.6–66.1] | 0.2324 |
Pirfenidone/Nintedanib | 29 (78.4%), 8 (21.6%) | 130 (57.8%), 95 (42.2%) | 102 (57.6%)/75 (42.4%) | 28 (58.3%)/ 20 (41.7%) | 0.0184 |
History of AE | 0 (0%) | 33 (14.7%) | 25 (14.1%) | 8 (16.7%) | 0.0067 |
Never/former & current smoker | 6 (16.2%), 31 (83.8%) | 46 (20.4%), 179 (79.6%) | 37 (20.9%)/140 (79.1%) | 9 (18.8%)/39 (81.3%) | 0.6602 |
Smoking pack-year | 40.0 [18.0–59.0] | 30.0 [3.0–46.0] | 30.8 [3.0–45.0] | 30.0 [7.5–48.0] | 0.1297 |
BMI, kg/m2 | 24.3 [21.3–25.3] | 22.9 [20.7–25.4] | 23.3 [21.4–25.7] | 21.9 [19.5–23.5] | 0.2329 |
Pulmonary function test | |||||
FVC, %-pred | 73.2 [62.4–83.7] | 67.7 [56.5–79.8] | 68.3 [58.5–80.2] | 61.4 [47.7–77.4] | 0.1018 |
FEV1, %-pred | 75.3 [67.6–87.6] | 74.4 [63.3–92.2] | 76.4 [64.5–90.9] | 73.1[55.8–90.9] | 0.7480 |
FEV1/FVC, % | 83.4 [79.9–88.9] | 86.4 [79.9–92.2] | 85.4 [79.8–91.4] | 90.2 [80.4–94.5] | 0.0438 |
DLCO, % | 62.2 [50.2–67.6] (n = 37) | 58.3 [43.4–72.0] (n = 185) Unable to perform (n = 14) | 58.4 [44.0–71.5] (n = 153) Unable to perform (n = 7) | 57.6 [38.1–77.7] (n = 32) Unable to perform (n = 7) | 0.3516 |
GAP stage, I, II, III | 18 (48.6%), 19 (51.4%), 0 (0%) | 76 (38.2%), 90 (45.2%), 33 (16.6%) | 63 (39.4%), 74 (46.3%), 23 (14.4%) | 13 (33.3%), 16 (41.0%), 10 (25.6%) | 0.0267 |
6-min walk test | |||||
Distances, m | 432 [345–515] (n = 28) | 400 [310–484] (n = 134) | 400 [308–484] (n = 116) | 400 [180–482] (n = 18) | 0.1990 |
Minimum SpO2 < 90% | 19/28 (67.9%) | 100/134 (74.6%) | 87/116 (75.0%) | 13/18 (72.2%) | 0.4845 |
UCG | |||||
TRV ≥ 2.9 m/s | 2 (8.7%) (n = 29) | 24 (17.9%) (n = 134) | 16 (15.4%) (n = 104) | 8 (26.7%) (n = 30) | 0.1719 |
Laboratory | |||||
Hb, g/dl | 14.0 [13.2–15.1] | 13.5 [12.3–14.7] | 13.6 [12.4–14.8] | 13.3 [12.1–14.7] | 0.0612 |
TP, g/dl | 7.5 [7.2–7.9] | 7.4 [6.9–7.8] | 7.5 [7.0–7.8] | 7.3 [6.7–7.8] | 0.0855 |
Alb, g/dl | 4.1 [4.0–4.2] | 3.9 [3.6–4.1] | 3.9 [3.6–4.1] | 3.7 [3.5–4.0] | 0.0006 |
LDH, U/L | 241 [210–270] | 230 [203–273] | 228 [203–268] | 244 [206–280] | 0.8415 |
CRP, mg/dl | 0.2 [0.1–0.5] | 0.3 [0.1–0.6] | 0.3 [0.1–0.6] | 0.3 [0.1–0.6] | 0.5156 |
KL-6, U/ml | 1124 [776–1473] | 1062 [798–1524] | 1057 [796–1560] | 1105 [817–1402] | 0.7003 |
SP-D, ng/ml | 232 [136–345] | 249 [165–370] | 247 [158–375] | 251 [188–369] | 0.7278 |
Treatment | |||||
None | 30 (81.1%) | 128 (56.9%) | 126 (71.2%) | 22 (45.8%) | 0.0060 |
LTOT | 6 (16.2%) | 85 (37.8%) | 65 (36.7%) | 20 (41.7%) | 0.0144 |
Flow rate during rest | |||||
< 1, 1–3, > 3, L/min | 4, 2, 0 | 40, 42, 3 | 30, 32, 3 | 10, 10, 0 | |
Immunosuppressants | 3 (8.1%) | 50 (22.2%) | 37 (20.9%) | 13 (27.1%) | 0.0486 |
PSL | 3 | 30 | 23 | 8 | |
PSL + CyA | 0 | 11 | 7 | 4 | |
PSL + CPA | 5 | 3 | 2 | ||
PSL + Tac | 4 | 4 | 0 |