Table 1

Clinical and demographic characteristics of the 706 patients from the EUSTAR database included in the analysis

CharacteristicsPatients (n=706)Available data (% patients)
Male sex172 (24.4)100
Age, mean±SD52.9±12.9100
Disease duration, months (mean±SD)101.1±94.094.1
Body weight, kg (mean±SD)64.6±13.497.2
Laboratory parameters
ANA positive657 (94.4)98.6
ACA positive48 (7.1)96.3
Anti-Scl70 positive414 (60.2)97.5
Anti-U1RNP positive27 (4.7)81.3
Creatine kinase elevation64 (9.5)95.2
Proteinuria57 (8.4)95.6
Hypocomplementaemia39 (6.3)88.1
ESR>20 mm/1 hour, mean±SD25.3±20.694.5
CRP elevation190 (27.7)97.0
Raynaud’s present683 (96.7)100
DU ever266 (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 month162 (23.3)98.3
Tendon friction rubs89 (12.8)98.3
Joint synovitis108 (15.4)99.3
Joint contractures310 (44.4)98.9
Muscle weakness164 (23.4)99.3
mRSS, mean±SD14.2±9.193.2
Worsening of skin changes within the last month141 (20.3)98.3
Skin progression rate, mean±SD0.6±1.788.2
Arterial hypertension154 (21.9)99.6
Pericardial effusion58 (8.9)92.5
Echocardiography-suspected PH113 (16.3)98.0
Conduction blocks104 (15.6)94.2
Abnormal diastolic function170 (25.0)96.2
Lung fibrosis†131 (19.7)94.3
Significant dyspnoea91 (13.2)97.7
DLCO, %predicted (mean±SD)64.1±20.294.1
FVC, %predicted (mean±SD)86.4±21.396.5
FEV1, %predicted (mean±SD)85.0±18.778.3
TLC, %predicted (mean±SD)84.2±19.966.1
LVEF, %predicted (mean±SD)61.7±7.096.5
Oesophageal symptoms455 (64.5)99.9
Stomach symptoms192 (27.4)99.3
Intestinal symptoms177 (25.2)99.3
Renal crisis34 (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.