Clinical, laboratory, and ongoing therapeutic characterization of the study population.
| Age, yrs, median (range) | 55 (22–77) |
| Male sex | 9 (20) |
| Height, cm, median (range) | 162 (150–187) |
| Years since diagnosis, median (range) | 10 (2–23) |
| ANA | 44 (100) |
| ACA | 15 (34) |
| Scl-70 | 24 (54) |
| RNAP-3 | 1 (3) |
| Anti-RNP | 4 (9) |
| NVC pattern early vs active vs late | 2 vs 10 vs 32 (5 vs 22.5 vs 72.5) |
| Limited/diffuse cutaneous subset | 32/12 (73–27) |
| Previous DU | 40 (91) |
| No. current DU, median (range) | 2 (1–7) |
| mRSS, median (range) | 9 (2–35) |
| Lung involvement | 27 (61) |
| FVC, median (range) | 91.3 (54–180) |
| DLCO, median (range) | 59.10 (38–102) |
| GI involvement | 28 (64) |
| Creatinine, mg/dl, median (range) | 0.80 (0.55–1.60) |
| Creatine kinase, mg/dl, median (range) | 94 (34–505) |
| PAH | 8 (18) |
| Scleroderma renal crisis | 1 (2) |
| Cardiac involvement | 11 (25) |
| Muscular involvement | 8 (18) |
| Intravenous iloprost | 26 (59) |
| Endothelin receptors antagonists | 18 (41) |
| Phosphodiesterase 5 inhibitors | 13 (31) |
| Calcium channel blockers | 14 (32) |
| Losartan | 16 (36) |
| Fluoxetine | 8 (18) |
| Mycophenolate mofetil | 9 (20) |
| Methotrexate | 8 (18) |
| Azathioprine | 1 (2) |
| Hydroxychloroquine | 7 (16) |
| Steroids < 7.5 mg/day prednisone equivalent | 19 (43) |
| Statins | 9 (20) |
Data are n (%) unless otherwise indicated. ACA: anticentromere antibodies; ANA: antinuclear antibodies; DU: digital ulcers; FVC: forced vital capacity; mRSS: modified Rodnan skin score; NVC: nailfold videocapillaroscopy; RNAP-3: anti-RNA polymerase III antibodies; Scl-70: antitopoisomerase I antibodies; GI: gastrointestinal; PAH: pulmonary arterial hypertension.