Table 1.
Clinical and demographic characteristics of the 706 patients from the EUSTAR database included in the analysis
| Characteristics | Patients (n=706) | Available data (% patients) |
| Demographic | ||
| Male sex | 172 (24.4) | 100 |
| Age, mean±SD | 52.9±12.9 | 100 |
| Disease duration, months (mean±SD) | 101.1±94.0 | 94.1 |
| Body weight, kg (mean±SD) | 64.6±13.4 | 97.2 |
| Laboratory parameters | ||
| ANA positive | 657 (94.4) | 98.6 |
| ACA positive | 48 (7.1) | 96.3 |
| Anti-Scl70 positive | 414 (60.2) | 97.5 |
| Anti-U1RNP positive | 27 (4.7) | 81.3 |
| Creatine kinase elevation | 64 (9.5) | 95.2 |
| Proteinuria | 57 (8.4) | 95.6 |
| Hypocomplementaemia | 39 (6.3) | 88.1 |
| ESR>20 mm/1 hour, mean±SD | 25.3±20.6 | 94.5 |
| CRP elevation | 190 (27.7) | 97.0 |
| Vascular | ||
| Raynaud’s present | 683 (96.7) | 100 |
| DU ever | 266 (38.1) | 98.9 |
| Active DU* | 126 (18.1) | 98.7 |
| Scleroderma (puffy fingers) | 303 (44.2) | 97.2 |
| Worsening of finger vascularisation within the last month | 162 (23.3) | 98.3 |
| Musculoskeletal | ||
| Tendon friction rubs | 89 (12.8) | 98.3 |
| Joint synovitis | 108 (15.4) | 99.3 |
| Joint contractures | 310 (44.4) | 98.9 |
| Muscle weakness | 164 (23.4) | 99.3 |
| Skin | ||
| mRSS, mean±SD | 14.2±9.1 | 93.2 |
| Worsening of skin changes within the last month | 141 (20.3) | 98.3 |
| Skin progression rate, mean±SD | 0.6±1.7 | 88.2 |
| Cardiopulmonary | ||
| Arterial hypertension | 154 (21.9) | 99.6 |
| Pericardial effusion | 58 (8.9) | 92.5 |
| Echocardiography-suspected PH | 113 (16.3) | 98.0 |
| Conduction blocks | 104 (15.6) | 94.2 |
| Abnormal diastolic function | 170 (25.0) | 96.2 |
| Lung fibrosis† | 131 (19.7) | 94.3 |
| Significant dyspnoea | 91 (13.2) | 97.7 |
| DLCO, %predicted (mean±SD) | 64.1±20.2 | 94.1 |
| FVC, %predicted (mean±SD) | 86.4±21.3 | 96.5 |
| FEV1, %predicted (mean±SD) | 85.0±18.7 | 78.3 |
| TLC, %predicted (mean±SD) | 84.2±19.9 | 66.1 |
| LVEF, %predicted (mean±SD) | 61.7±7.0 | 96.5 |
| Gastrointestinal | ||
| Oesophageal symptoms | 455 (64.5) | 99.9 |
| Stomach symptoms | 192 (27.4) | 99.3 |
| Intestinal symptoms | 177 (25.2) | 99.3 |
| Kidney | ||
| Renal crisis | 34 (4.8) | 99.4 |
| Disease activity | ||
| Active disease‡ | 191 (30.7) | 88.1 |
Data are n (%) unless otherwise stated. (Percentages with characteristics were calculated from numbers of patients with data available).
Clinical manifestations were defined according to the EUSTAR definitions.15
Presence of significant dyspnoea was based on the judgement of the treating physician.
*Active DUs was a composite endpoint that was considered positive if either DU (from the minimal essential dataset) or digital gangrene was present.
†Lung fibrosis was defined as FVC<60% or FVC<70% and presence of lung fibrosis on high-resolution computed tomography.
‡Active disease was defined as score >3 calculated according to the EScSG disease activity indices for SSc.38
ACA, anti-centromere antibody; ANA, anti-nuclear antibody; CRP, C-reactive protein; DLCO, diffusion capacity of the lung for carbon monoxide; DU, digital ulcer; ESR, erythrocyte sedimentation rate;EScSG, European Scleroderma Study Group; EUSTAR, European Scleroderma Trials and Research; FEV1, forced expiratory volume after 1 s; FVC, forced vital capacity; LVEF, left ventricular ejection fraction;mRSS, modified Rodnan skin score; PH, pulmonary hypertension; TLC, total lung capacity.