Disease characteristics and severity criteria of the patients at the time of inclusion
| Organ involvement | No/No evaluable patients | % |
|---|---|---|
| Severity criteria | ||
| VC, vital capacity; Tlco, carbon monoxide transfer factor; LVEF, left ventricular ejection fraction. | ||
| Skin | 57 | 100 |
| Skin score >50% maximum | 27/43 | 63 |
| Skin score (% of maximum), median (range) | 56.5 (18–85) | |
| Lung | ||
| VC <70% of body mass related mean | 27/47 | 57 |
| Tlco <70% of body mass related mean | 30/47 | 64 |
| Pulmonary hypertension >30 mm Hg | 10/32 | 31 |
| Pulmonary artery pressure, median (range) | 23 (12–64) | |
| Cardiovascular symptoms | ||
| Raynaud’s phenomenon | 45/54 | 83 |
| Arterial hypertension | 5/53 | 9 |
| LVEF <45% normal | 1/42 | 2 |
| Kidney | ||
| Creatinine clearance (ml/min) | ||
| >100 | 7/19 | 37 |
| >70 and <100 | 12/19 | 63 |
| Digestive tract | ||
| Oesophageal | 36/52 | 69 |
| Gastrointestinal | 9/53 | 17 |
| Serum autoantibodies | 30/50 | 60 |
| Anti-Scl-70 antibodies (DNA topoisomerase I) | 7/47 | 15 |
| Anticentromere antibodies | ||