Table 2

 Disease characteristics and severity criteria of the patients at the time of inclusion

Organ involvementNo/No evaluable patients%
Severity criteria
VC, vital capacity; Tlco, carbon monoxide transfer factor; LVEF, left ventricular ejection fraction.
Skin 57100
Skin score >50% maximum27/4363
Skin score (% of maximum), median (range)56.5 (18–85)
VC <70% of body mass related mean27/4757
Tlco <70% of body mass related mean30/4764
Pulmonary hypertension >30 mm Hg10/3231
Pulmonary artery pressure, median (range)23 (12–64)
Cardiovascular symptoms
Raynaud’s phenomenon45/5483
Arterial hypertension5/539
LVEF <45% normal1/422
Creatinine clearance (ml/min)
    >70 and <10012/1963
Digestive tract
Serum autoantibodies 30/5060
Anti-Scl-70 antibodies (DNA topoisomerase I)7/4715
Anticentromere antibodies