Table 2.
Characteristics of the AE-IPF Patients.
| Survived patients (n=28) | Fetal patients (n=19) | p value | |
|---|---|---|---|
| Median age (years old) (range) | 75 (58-86) | 74 (68-85) | 0.914 |
| Never smoker (%) | 4 (15%) | 1 (5%) | 0.635 |
| Male (%) | 24 (86%) | 18 (95%) | 0.635 |
| Pulmonary function before AE-IPF | |||
| %FVC | 77±19 | 73±18 | 0.575 |
| %FEV1.0 | 102±22 | 91±22 | 0.162 |
| %DLCO | 52±17 | 55±14 | 0.571 |
| Treatment before AE-IPF | |||
| CS | 3 (11%) | 5 (26%) | 0.240 |
| Cyclosporine A | 1 (4%) | 2 (11%) | 0.557 |
| Pirfenidone | 5 (18%) | 3 (16%) | 1.000 |
| N-acetylcysteine | 12 (43%) | 6 (32%) | 0.546 |
| No medication | 14 (50%) | 8 (42%) | 0.767 |
| Respiratory failure before AE-IPF | 4 (14%) | 5 (26%) | 0.453 |
| Clinical parameters at AE-IPF onset | |||
| White blood cell (/μL) | 9,800±3,200 | 11,000±2,900 | 0.590 |
| C-reactive protein (mg/dL) | 7.3±5.9 | 8.2±4.6 | 0.174 |
| LDH (IU/L) | 336±70 | 378±145 | 0.198 |
| KL-6 (U/mL) | 1,509±1,135 | 1,418±1,026 | 0.832 |
| SP-D (ng/mL) | 321±201 | 437±348 | 0.144 |
| SP-A (ng/mL) | 91±39 | 87±46 | 0.074 |
| PaO2/FiO2 ratio | 277±88 | 194±111 | 0.008* |
| Medications for AE-IPF | |||
| CS pulse therapy | 27 (100%) | 19 (100%) | 1.000 |
| CS maintenance therapy | 27 (100%) | 19 (100%) | 1.000 |
| Cyclosporine A | 23 (85%) | 15 (79%) | 1.000 |
| Recombinant thrombomodulin | 17 (63%) | 7 (37%) | 0.143 |
| Hypoxemia after AE-IPF | |||
| At rest | 8 (29%) | - | - |
| On exertion | 9 (32%) | - | - |
| Degree of lowering of BI after AE-IPF | |||
| 0 | 6 (21%) | - | - |
| mild (5-15) | 10 (36%) | - | - |
| severe (20-) | 12 (43%) | - | - |
| Median Survival duration after AE-IPF onset (days) (range) | 13 (2-82) | - |
*p<0.01.
AE: acute exacerbation, IPF: idiopathic pulmonary fibrosis, FVC: forced vital capacity, FEV1.0: forced expiratory volume in one second, DLCO: diffusing capacity of the lung for carbon monoxide, CS: corticosteroids, LDH: lactate dehydrogenase