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 |