Table 1.
Characteristics of the 60 patients with systemic sclerosis included in the discovery cohort
| Age (years), median (range) | 53.5 (28-81) |
| Disease duration (years), median (range) | 9 (1-50) |
| Limited/Diffuse cutaneous subset, n (%) | 36 (60)/24 (40) |
| Modified Rodnan skin score > 14, n (%) | 19 (32) |
| History of digital ulcers, n (%) | 29 (47) |
| Pulmonary fibrosis on CT scan, n (%) | 28 (47) |
| Pulmonary arterial hypertension on RHC, n (%) | 4 (7) |
| Positive antinuclear antibodies (> 1/160), n (%) | 53 (88) |
| Positive antitopoisomerase-1 antibodies, n (%) | 28 (47) |
| Positive anticentromere antibodies, n (%) | 12 (20) |
| FVC < 75% predicted, n (%) | 13 (22) |
| DLCO/AV < 75% predicted, n (%) | 23 (38) |
| Treatment with calcium channel blockers, n (%) | 60 (100) |
| Treatment with angiotensin-converting enzyme inhibitors, n (%) | 19 (32) |
| Treatment with endothelin-receptor antagonists and/or PDE5 inhibitors, n (%) | 12 (20) |
DLCO/AV, diffusing capacity for carbon monoxide/alveolar volume; FVC, forced vital capacity; PDE5, phosphodiesterase type 5; RHC, right heart catheterization.