Table 1.
Clinical characteristics, severity of IPF, and treatments in all patients with AE-IPF.
| n = 39 episodes, median (range) | |
|---|---|
| Age, years | 69 (50, 84) |
| Sex, male/female | 37/2 |
| Smoking, never/ex/current | 3/32/4 |
| Smoking pack years at first AE | 35 (0, 80) |
| Period from IPF-diagnosis to AE, mo | 75 (0, 205) |
| Observation period, mo | 62 (2, 205)* |
| The number of AEs, 1/2 | 32/7 |
| BMI | 21.9 (16.3, 28.9) |
| Body surface area, m2 | 1.59 (1.38, 1.91) |
| Peripheral blood WBC at AE, /μL | 9755 (1900, 20000) |
| Peripheral blood neutrophils at AE, /μL | 6954 (1539, 18200) |
| CRP at AE, mg/dL | 7.4 (0.9, 23.7) |
| Serum LDH at AE, IU/L | 347 (183, 693) |
| Serum KL-6 at AE, U/mL | 1535 (481, 6404) |
| Serum SP-D at AE, ng/mL | 338 (23, 1330) |
| P/F ratio at AE | 176 (38, 386) |
| HRCT extent scores at AE (full score: 25) | 21 (13, 25) |
| Period from admission to the beginning of AE treatment, days | 1 (0, 17) |
| Administration of steroid pulse therapy at AE, +/− | 40/0 |
| Administration of Immunosuppressants at AE, +/− | 28/11 |
| Data before AE** | |
| FVC, % pred | 57.6 (37.5, 89.3) |
| DLCO, % pred | 57.1 (33.5, 85.9) |
| PaO2 at rest, Torr | 72.8 (49.0, 91.0) |
| HRCT extent scores (full score: 25) | 12.5 (7, 19) |
| Distance in 6MWT, mo | 370 (160, 507) |
| Minimum SpO2 in 6MWT, % | 82 (60, 95) |
|
JRS severity grade of interstitial pneumonia, I/II/III/IV/unknown |
9/0/12/14/4 |
|
The GAP staging system, I/II/III/unknown |
7/13/14/5 |
| Preceding treatments for IPF, +/− | 24/15 |
| Preceding oxygen therapy, +/− | 13/26 |
*Observation period in 32 patients with IPF.
**Pulmonary function tests, severity scores, HRCT, and serum markers were evaluated within 12 months before AE-IPF.
Abbreviations; AE: acute exacerbation, BMI: body mass index, FVC: forced vital capacity, DLCO: diffusion lung capacity for carbon monoxide, PaO2: partial pressure of arterial oxygen, HRCT: high-resolution computed tomography, 6MWT: 6-minute walk test, SpO2: partial oxygen saturation, LDH: lactate dehydrogenase, KL-6: Krebs von den Lungen-6, SP-D: surfactant protein D, JRS: Japanese respiratory society, GAP: gender, age, and physiology, IPF: idiopathic pulmonary fibrosis, WBC: white blood cell, CRP: c-reactive protein, P/F: PaO2/FiO2.