Table 1.
Baseline characteristics
| Patients | Controls | p | |
|---|---|---|---|
| No of patients, n | 24 | 157 | |
| Age (year) | 66 ± 6 | 50 ± 17 | < 0.001 |
| Sex (female), n (%) | 5 (21) | 75 (48) | 0.02 |
| Height (cm) | 171 ± 8 | 170 ± 9 | 0.26 |
| Weight (kg) | 77 ± 16 | 72 ± 13 | 0.16 |
| Body mass index (kg/m2) | 25.6 ± 3.7 | 24.8 ± 3.7 | 0.22 |
| Smoking history, n (%) | 21 (87) | ||
| Pack-years | 24 ± 16 | ||
| Comorbidities, n (%) | |||
| Diabetes | 5 (21) | ||
| High blood pressure | 4 (17) | ||
| Cardiovascular disease | 11 (46) | ||
| UIP pattern on CT-scan, n (%) | |||
| Definite | 12 (50) | ||
| Probable | 11 (46) | ||
| Unclassifiable | 1 (4) | ||
| Pulmonary hypertension, n (%) | 8 (33) | ||
| sPAP (mmHg) | 38 [33; 45] | ||
| mPAP (mmHg) | 27 ± 6 | ||
| PVR (Wood) | 2.9 [2.1; 4.2] | ||
| Dyspnea (mMRC score) | 2 [1; 2] | ||
| O2 supplementation, n (%) | 8 (33) | ||
| Specific fibrosis therapy, n (%) | 21 (87) | ||
| Nintedanib, n (%) | 17 (77) | ||
| Pirfenidone, n (%) | 19 (83) | ||
| Systemic corticosteroids, n (%) | 4 (17) | ||
| Dosing (mg) | 7.5 [5; 22] | ||
| Lung function test | |||
| FEV1 (L) | 2.3 ± 0.6 | 3.2 ± 0.9 | < 0.001 |
| FEV1 (% pred) | 78 ± 20 | 100 ± 14 | < 0.001 |
| FVC (L) | 2.7 ± 0.8 | 4.0 ± 1.1 | < 0.001 |
| FVC (% pred) | 72 ± 21 | 103 ± 12 | < 0.001 |
| FEV1/FVC ratio (%) | 84 ± 6 | 81 ± 6 | 0.02 |
| TLC (L) | 4.4 ± 1.0 | ||
| TLC (% pred) | 69 ± 15 | ||
| DLCO (% pred) | 41 ± 12 | ||
| Six minutes walking test (m) | 524 [455; 553] | ||
| Blood biology | |||
| Albumin (g/L) | 41 [38; 44] | ||
| CPK (IU/L) | 79 ± 33 | ||
| Creatinine (µmol/L) | 71 ± 18 | ||
| CRP (mg/L) | 3 [2; 9] | ||
| ANA, n (%) | 15 (62) | ||
| ANCA, n (%) | 2 (9) | ||
| Time between diagnosis and PFT (days) | 1604 ± 1131 | ||
| Time between diagnosis and US (days) | 1632 ± 1137 | ||
| Time between PFT and US (days) | 13 [0; 64] | ||
| Time between diagnosis and CT (days) | 1555 ± 1147 | ||
| Time between PFT and CT (days) | 0 [-97; 3] | ||
UIP usual interstitial pneumonia, PAP pulmonary arterial pressure, PVR pulmonary vascular resistance, mMRC modified Medical Research Council, ANA antinuclear antibody, PFT pulmonary function test, US ultrasound