Definition of some of the items recorded in the clinical chart
| 1 | Skin score: recorded according to Kahaleh et al.19 This score is evaluated on a four point scale (0 for normal skin, 1 for thickened skin, 2 for thickened, unpinchable skin, 3 for hidebound skin) at 22 body regions (maximal value 66) |
| 2 | Oesophageal hypomotility:radiologically documented hypomotility |
| 3 | Interstitial lung disease: signs of lung fibrosis documented by chest x ray and/or computed tomography (CT) scan. The standard chestx ray was scored 0–3: 0 = absence of interstitial lung disease; 1 = bibasilar lung fibrosis; 2 = diffuse interstitial lung fibrosis; 3 = honey combing.20 CT was scored 0–3: 0 = absence of interstitial lung disease; 1 = ground glass appearance; 2 = subpleural septal lines; 3 = honey combing. Test of pulmonary function (forced vital capacity) and carbon monoxide transfer factor were performed, as well |
| 4 | Cardiac disease: signs of ischaemia and/or myocardial fibrosis or pericarditis detected by ECG and/or standard echocardiography. Holter ECG was performed to detect dysrhythmias |
| 5 | Kidney disease: proteinuria, haematuria, or decreased creatinine clearance, even in the absence of a typical scleroderma renal crisis |