Table 2

Disease characteristics of the European League Against Rheumatism Scleroderma Trials and Research cohort

TotalMD (%)lcSScdcSScP Value (lcSSc vs dcSSc)Other
Disease characteristics
ACR criteria fulfilled, %1000100100100
Age at onset of RP (years), mean (SD)42.2 (14.9)3.842.1 (15.0)42.2 (14.7)NS41.1 (14.6)
Age at onset of first non-RP (years), mean (SD)45.9 (14.2)10.447.2 (14.1)44.2 (14.2)****43.9 (14.5)
Difference between onset of RP and onset of first non-RP (years), mean (SD)3.8 (8.1)11.95.1 (9.1)1.8 (5.5)****2.9 (8.1)
RP (ever), %96.30.996.696.1NS93.1
Digital ulcers (ever),%36.00.432.742.4****22.9
Digital ulcers (active), %16.92.315.520.1*5.6
Joint synovitis, %15.70.712.720.0****21.1
Joint contractures, %32.10.621.948.7****27.5
Tendon friction rubs, %****7.9
Muscle weakness, %****36.5
Muscle atrophy, %****19.7
Skin involvement
mRSS, median (IQR)8 (4–15)6.16 (4–10)16 (10–24)****3 (2–8)
Face, median110.5010
Left fingers, median29.9221
Right fingers, median29.9221
Left hand, median110.3010
Right hand, median110.4010
Left lower arm, median010.5010
Right lower arm, median010.8010
Left upper arm, median010.7000
Right upper arm, median010.8000
Chest, median010.8000
Abdomen, median011000
Left upper leg, median010.7000
Right upper leg, median010.8000
Left lower leg, median010.7000
Right lower leg, median010.7000
Left foot, median010.6000
Right foot, median010.7000
Laboratory parameters
ANA positive, %
ACA positive, %32.35.648.27.2****27.6
Scl70 positive, %36.85.323.259.8****19.6
U1RNP positive, %7.728.36.68.1NS21.7
RNA polymerase III positive, %2.449.91.24.7**2.9
CRP elevation, %****25.0
ESR, mean (SD)25.0 (21.2)12.123.2 (19.9)27.7 (22.9)****29.1 (23.7)
CK elevation, %****16.7
Proteinuria, %****10.7
Hypocomplementaemia, %5.816.65.28.8*21.7
Organ involvement
Dyspnoea, %35.30.632.240.5****35.3
NYHA, median19.811NS1
Pulmonary arterial hypertension, %21.15.520.722.1NS19.3
PAP, systolic (mm Hg, measured on echocardiogram), mean (SD)31.7 (12.4)34.531.5 (12.5)31.9 (12.1)NS34.3 (12.1)
Lung fibrosis (on plain x-ray), %39.58.331.352.0****44.9
Lung fibrosis (on HRCT), %51.936.943.564.1****36.8
Ground-glass opacification (on HRCT), %29.836.922.040.3****26.3
Restrictive defect (lung function test), %32.06.723.345.9****30.6
TLCO, lung function test (% predicted), mean (SD)68.3 (21.1)34.070.9 (21.0)63.9 (20.6)****69.2 (21.0)
FVC, lung function test (% predicted), mean (SD)92.2 (21.3)21.296.9 (20.2)84.0 (20.3)****96.9 (26.5)
FEV-1, lung function test (% predicted), mean (SD)90.0 (19.4)33.293.5 (18.8)84.1 (18.6)****93.3 (22.3)
TLC, lung function test (% predicted), mean (SD)89.5 (19.2)48.693.0 (17.4)83.8 (20.6)****95.1 (16.8)
Gastrointestinal tract
Oesophageal symptoms, %67.30.366.469.5**62.1
Stomach symptoms, %24.10.622.427.1****20.3
Intestinal symptoms, %23.50.423.224.1NS23.2
Cardiovascular system
Pulse, mean (SD)77.3 (13.6)13.676.0 (13.4)79.5 (13.8)****75.1 (10.9)
Arterial hypertension, %20.60.621.220.3NS16.4
Palpitations, %****31.9
Conduction blocks, %11.04.710.112.2**13.4
Left ventricular ejection fraction (%, measured on echocardiogram), mean (SD)62.6 (6.3)22.562.5 (5.8)62.7 (7.1)*63.4 (5.5)
Diastolic function abnormal, %17.46.517.218.0NS16.8
Pericardial effusion, %8.623.16.411.9****7.3
Renal crisis, %****0.7
Worsening of skin within the past month, %21.92.314.434.4****14.8
Worsening of finger vascularisation within the past month, %25.02.321.530.7****24.1
Worsening of cardiopulmonary manifestations within the past month, %13.92.810.719.5****9.3
Active disease defined by Valentini et al 19 (total count >3), %32.642.022.951.1****12.5
Nailfold capillaroscopy
Scleroderma pattern present, %90.959.190.192.2NS87.5
Scleroderma pattern66.3****
Early pattern, %24.026.719.336.4
Active pattern, %39.643.234.245.5
Late pattern, %36.430.146.518.2
  • * p<0.05;

  • ** p<0.01;

  • *** p<0.001;

  • newly introduced items (available since online documentation has started) are highlighted in bold (find additional patient characteristics in online supplementary table S2).

  • ACA, anticentromere antibodies; ACR, American College of Rheumatology; ANA, antinuclear antibodies; CK, creatine kinase; CRP, C-reactive protein; dcSSc, diffuse cutaneous systemic sclerosis; ESR, erythrocyte sedimentation rate; FEV-1, forced expiratory volume in one second; FVC, forced vital capacity; HRCT, high-resolution CT; lcSSc, limited cutaneous systemic sclerosis; MD, missing data; mRSS, modified Rodnan skin score; NA, not applicable; NS, not significant; NYHA, New York Heart Association; PAP, pulmonary arterial pressure; RNA, ribonucleic acid; RP, Raynaud's phenomenon; Scl70, antitopoisomerase I antibodies; TLC, total lung capacity; TLCO, carbon monoxide transfer factor; U1RNP, uridine-rich ribonucleic protein.