Table 1

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

CharacteristicsPatients (n=706)Available data (% patients)
Demographic
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
Vascular
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
Musculoskeletal
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
Skin
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
Cardiopulmonary
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
Gastrointestinal
Oesophageal symptoms455 (64.5)99.9
Stomach symptoms192 (27.4)99.3
Intestinal symptoms177 (25.2)99.3
Kidney
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.