Table 4

 Prevalence of disease presentation according to autoantibody serology

ANA positiveScl70 positiveACA positivep (Scl70 vs ACA)
ACA, anticentromere autoantibody; ANA, antinuclear antibodies; CK, creatine kinase; DLCO, diffusion capacity of the lung for carbon monoxide; dcSSc, diffuse cutaneous systemic sclerosis; lcSSc, limited cutaneous systemic sclerosis; LVEF, left ventricular ejection fraction; PAH, pulmonary artery hypertension (assessed by echocardiography); RO, onset of Raynaud’s phenomenon.
Number of patients334613301106<0.001
Presenting as dcSSC37.1%60.0%7.3%<0.001
Presenting as lcSSC57.4%36.1%88.7%<0.001
Presenting as “other”5.5%3.9%4.0%0.88
Women87.3%83.7%94.4%<0.001
Age (years), mean (SD)55.1 (13.6)52.6 (13.7)59.6 (11.8)<0.001
Age at RO (years), mean (SD)42.7 (14.6)42.2 (14.4)43.4 (14.7)0.28
Age at first non-RO (years), mean (SD)46.4 (13.8)44.5 (14.0)50.0 (12.6)<0.001
Time between RO and non-RO (years), mean (SD)3.7 (7.6)2.4 (5.6)6.5 (10.0)<0.001
mRSS (years), mean (SD)12.0 (9.1)15.1 (9.9)8.2 (5.9)<0.001
Active disease32.7%45.2%18.9%<0.001
Elevated acute-phase reactants31.9%42.6%20.7%<0.001
Raynaud’s phenomenon96.3%97.4%96.7%0.45
Digital ulcers36.7%44.8%31.2%<0.001
Synovitis16.7%21.4%11.9%<0.001
Joint contractures (any joint)33.7%44.5%17.6%<0.001
Tendon friction rubs13.1%18.9%6.0%<0.001
Muscle weakness28.4%32.2%22.7%<0.001
Muscle atrophy14.6%16.1%9.5%<0.001
CK elevation7.6%8.7%2.9%<0.001
Oesophagus67.9%68.0%70.7%0.18
Stomach24.5%24.1%26.9%0.11
Intestine22.5%20.7%25.1%0.01
Pulmonary fibrosis42.6%60.2%21.3%<0.001
Lung restrictive defect35.8%50.3%17.4%<0.001
% of predicted DLCO (years), mean (SD)68.9 (21.6)65.1 (20.9)75.0 (20.9)<0.001
PAH21.1%23.2%22.0%0.36
    PAH without fibrosis8.0%5.0%13.0%<0.001
    PAH with fibrosis12.7%17.2%8.0%<0.001
Dyspnoea38.6%44.5%29.4%<0.001
Palpitations24.8%27.2%23.2%0.01
Conduction block11.2%13.6%9.1%<0.001
Diastolic dysfunction15.7%17.7%12.7%0.001
Reduced LVEF5.7%5.9%5.2%0.29
Hypertension18.5%14.4%20.0%<0.001
Hypertensive renal crisis2.3%2.0%1.3%0.15
Proteinuria6.0%7.8%2.7%<0.001