Demographics and clinical characteristics at inclusion in 190 patients with SSc. All values are n (%) unless otherwise specified.
| Patient Characteristics | |
|---|---|
| Demographics | |
| No. patients | 190 (100) |
| Female | 132 (69.5) |
| Age at inclusion, yrs, mean ± SD | 53 ± 15 |
| Clinical characteristics | |
| Age at diagnosis of SSc, mean ± SD yrs | 43 ± 15 |
| Disease form | |
| Limited SSc | 109 (57.4) |
| Diffuse SSc | 81 (42.6) |
| Time since RP occurrence, yrs, mean ± SD (IQR) | 14 ± 12 (5–20) |
| Time since first non-RP symptom, yrs, mean ± SD (IQR) | 9.7 ± 8.4 (3–14) |
| Organ involvement | |
| Any organ involvement | 160 (84.2) |
| Pulmonary fibrosis | 86 (45.3) |
| Pulmonary arterial hypertension* | 22 (11.6) |
| History of renal crisis | 6 (3.2) |
| Esophagus involvement | 140 (73.7) |
| Gastrointestinal involvement | 52 (27.4) |
| Skin involvement | |
| Modified Rodnan Skin Score, mean ± SD | 14.2 ± 8.8 |
| ≤ 14 | 108 (56.8) |
| > 14 | 81 (42.6) |
| Score not available | 1 (0.5) |
| Factors limiting hand function | |
| Nonulcerative calcinosis | 27 (14.2) |
| Tendinous retractions | 81 (42.6) |
| Ankylosis of the distal interphalangeal joints | 128 (67.4) |
| Ankylosis of the proximal interphalangeal joints | 107 (56.3) |
| Ankylosis of the metacarpophalangeal joints | 61 (32.1) |
| Immunological characteristics | |
| Antitopoisomerase 1 (Scl-70) antibodies | 97/188 (51.6) |
| Anticentromere antibodies | 54/181 (29.8) |
| Treatment for DU | |
| Bosentan | 190/190 (100) |
| Calcium channel blockers | 42/69 (60.9) |
| Iloprost | 35/190 (18.4) |
↵* Determined by either right heart catheterization with a mean pulmonary pressure > 25 mm Hg or Doppler echocardiography. DU: digital ulcer; IQR: interquartile range; RP: Raynaud phenomenon; SSc: systemic sclerosis.