Table 1. Patients characteristics.
| Characteristics | Control group | IPF group | IPF-AE group | NSIP group |
|---|---|---|---|---|
| Number | 14 | 16 | 9 | 8 |
| GAP stage I/II/III | – | 9 (56%)/7 (44%)/0 | N.D. | 8 (100%)/0/0 |
| Age | 67.0 (53.8–73.3) | 65.5 (63.3–67.0) | 75.0 (66.0–77.5)d,e | 52.0 (44.5–63.8) |
| Male/female | 5 (36%)/9 (64%) | 12 (75%)/4 (25%) | 9 (100%)/0b,e | 3 (38%)/5 (63%) |
| Smoker | 8 (57%) | 10 (63%) | 6 (67%) | 3 (38%) |
| Number of death | 0 | 7 (44%)a | 9 (100%)b,d,e | 5 (63%)c |
| Cause of death | ||||
| AE | 0 | 5 (31%) | 9 (100%) | 2 (25%) |
| Infection | 0 | 1 (6%) | 0 | 1 (13%) |
| Cancer | 0 | 1 (6%) | 0 | 2 (25%) |
| Complicating AE | – | 8 (50%) | 9 (100%) | 4 (50%) |
| Overall survival (days) | – | 973.0 (283.5–1,831.5) | – | 2,021.5 (800.3–2,650.0) |
| Time-to-AE (days) | – | 1,051.0 (407.0–1,900.8) | – | 1,220.0 (325.8–2,894.3) |
| Baseline blood data | ||||
| KL-6 (IU/mL) | N.D. | 1,138.0 (691.0–1,801.0) | 1,487.0 (989.0–2,176.0) | 1,972.0 (458.0–3,190.0) |
| SP-D (ng/mL) | N.D. | 286.5 (85.2–377.8) | 509.0 (399.0–613.0) | N.D. |
| PaO2 (Torr) | N.D. | 81.4 (73.9–88.3) | 50.1 (48.3–73.3)d | 85.5 (78.3–109.7) |
| Baseline pulmonary function | ||||
| FVC (%) | N.D. | 85.1 (54.5–99.7) | 47.6 (42.9–57.7) | 75.1 (67.1–89.0) |
| DLCO/VA (%) | N.D. | 83.6 (69.8–88.5) | 61.2 (46.6–66.4)d | 89.9 (67.0–114.7) |
| Treatment | ||||
| Corticosteroid | ||||
| Oral corticosteroid | 0 | 9 (56%)a | 5 (56%)b | 8 (100%)c |
| Methylprednisolone | 0 | 6 (38%) | 9 (100%)b,d,f | 3 (38%) |
| Immunosuppressant | ||||
| Cyclosporin A | 0 | 6 (38%) | 4 (44%) | 3 (38%) |
| Cyclophosphamide | 0 | 1 (6%) | 2 (22%) | 1 (13%) |
| Anti-fibrotic drug | ||||
| Pirfenidone | 0 | 6 (38%) | 1 (11%) | 0 |
| Nintedanib | 0 | 4 (25%) | 2 (22%) | 0 |
Data were expressed as the median [25th to 75th percentiles of the interquartile range (IQR)], unless otherwise stated. a, P<0.05 control versus IPF group; b, P<0.05 control versus IPF-AE group; c, P<0.05 control versus NSIP group; d, P<0.05 IPF versus IPF-AE group; e, P<0.05 NSIP versus IPF-AE group. The significant level was adjusted by Bonferroni’s correction. NSIP, nonspecific interstitial pneumonia; GAP, gender age physiology; IPF, idiopathic pulmonary fibrosis; AE, acute exacerbation; FVC, forced vital capacity; SP-D, surfactant protein D; DLCO/VA, diffusing capacity of the lung for carbon monoxide divided by the alveolar volume; N.D., no data.