Table 1. Patients’ characteristics at baseline (n = 54).
| Age | 52.6 ± 11.8 |
| Gender (male) | 29 (53.7%) |
| Radiological stage: | |
| I | 13 (24.1%) |
| II | 25 (46.3%) |
| III | 13 (24.1%) |
| IV | 3 (5.6%) |
| Radiological pulmonary involvement (stages III + IV) | 16 (29.6%) |
| Manifestation of disease | |
| predominantly pulmonary | 31 (57.4%) |
| predominantly extrapulmonary | 3 (5.6%) |
| Mixed | 20 (37.0%) |
| DLCO | |
| ≥ 80% | 19 (15.7%) |
| 60–80% | 27 (22.3%) |
| 40–60% | 5 (4.1%) |
| < 40% | 2 (1.7%) |
| FVC | |
| ≥ 80% | 39 (32.2%) |
| 60–80% | 10 (8.3%) |
| 40–60% | 3 (2.5%) |
| < 40% | 0 (0.0%) |
| ACE or sIL-2R elevated | 24 (44.4%) |
| OCS | 33 (61.1%) |
| PDGF-AB [pg/ml] | 1.99 ± 0.72 |
| VEGF [pg/ml] | 86.08 ± 41.86 |
| FGF-2 [pg/ml] | 1.82 ± 0.26 |
| TGF-β1 [ng/ml] | 23.86 ± 11.03 |
Data are presented as n (%) or mean ± SD.
ACE = Angiotensin converting enzyme, elevated >85 U/l. sIL2-R = soluble interleukin-2-receptor, elevated >730 U/ml. DLCO = diffusion capacity for carbon monoxide. FVC = forced vital capacity. OCS = Oral corticosteroid treatment. PDGF-AB = platelet-derived growth factor-AB. VEGF = vascular endothelial growth factor. FGF-2 = fibroblast growth factor-2. TGF- β1 = transforming growth factor-β1.