Characteristics of the patients with SSc included in the weighting and validation cohort study
| Characteristics | Overall | % of missingness |
| Age, years, median (IQR) | 57 (48–65) | 1.1 |
| Female gender (n, %) | 396 (84.8) | 1.1 |
| Time since RP onset, years, median (IQR) | 11 (5.8–20) | 26.3 |
| Time since first non-RP manifestations, years, median (IQR) | 9 (4.7–15) | 5.5 |
| Diffuse cutaneous SSc (n, %) | 152 (37.5) | 14.2 |
| Limited cutaneous SSc (n, %) | 253 (62.5) | 14.2 |
| mRSS, median (IQR) | 4 (0–8) | 26.5 |
| Presence of Raynaud’s phenomenon (n, %) | 332 (94.6) | 25.6 |
| Digital ulcers (n, %) | 47 (13) | 23.5 |
| Joint contractures (n, %) | 124 (35.7) | 26.5 |
| Joint synovitis (n, %) | 15 (4.4) | 28.4 |
| Oesophageal symptoms (dysphagia, reflux) (n, %) | 232 (60.3) | 18.4 |
| Stomach symptoms (early satiety, vomiting) (n, %) | 61 (17.6) | 26.5 |
| Intestinal symptoms (diarrhoea, bloating, constipation) (n, %) | 135 (33.8) | 15.5 |
| Malabsorption syndrome (n, %) | 18 (7.4) | 48.7 |
| Dyspnoea, NYHA stages III and IV (n, %) | 27 (9.6) | 40.7 |
| FVC, % predicted, median (IQR) | 95 (82–108) | 40.5 |
| FVC <80% predicted (n, %) | 58 (20.6) | 40.5 |
| DLCO/SB, % predicted, median (IQR) | 69 (55–81) | 44.9 |
| DLCO/SB, <70% predicted (n, %) | 133 (51.2) | 44.9 |
| Lung fibrosis detected by HRCT (n, %) | 78 (42.6) | 61.2 |
| Pulmonary hypertension (n, %) | 19 (6.6) | 39.4 |
| PAPsys, mm Hg, median (IQR) | 28 (24–32) | 54.4 |
| LVEF, %, median (IQR) | 60 (55–65) | 35.4 |
| ANA positive (n, %) | 319 (96.7) | 30.1 |
| ACA positive (n, %) | 118 (36.5) | 31.6 |
| Anti-Scl-70 AB positive (n, %) | 112 (35.2) | 32.6 |
| Anti-RNA Polymerase III AB positive (n, %) | 21 (7.6) | 41.1 |
| ESR, mm/h, median (IQR) | 17 (10–30) | 25.2 |
| CRP, mg/L, median (IQR) | 2 (0.9–5) | 35 |
| Immunosuppression (n, %) | 59 (21.2) | 41.1 |
Definitions of organ manifestations according to EUSTAR.19
ACA, anticentromere antibodies; ANA, antinuclear antibodies; CRP, C reactive protein; DLCO/SB, diffusing capacity of the lung for carbon monoxide/single breath; ESR, erythrocyte sedimentation rate; EUSTAR, European Scleroderma Trials And Research; FVC, forced vital capacity; HRCT, high resolution CT; LVEF, left ventricular ejection fraction; mRSS, modified Rodnan Skin Score; NYHA, New York Heart Association; RP, Raynaud’s phenomenon; Scl70, anti-Scl70 antibodies, anti-topoisomerase I antibodies; SSc, systemic sclerosis.