Table 3

 Prevalence of disease presentation according to the onset of Raynaud’s phenomenon

DcSScp (early vs late)lcSScp (early vs late)
Early RaynaudLate RaynaudEarly RaynaudLate Raynaud
ACA, anticentromere autoantibody; ANA, antinuclear antibodies; dcSSc, diffuse cutaneous systemic sclerosis; lcSSc, limited cutaneous systemic sclerosis; mRSS, modified Rodnan Skin Score; NA, not applicable; PAH, pulmonary artery hypertension (assessed by echocardiography).
Manifestations with statistically similar prevalence between early and late onset are not shown.
Number of patients553594NA9141003NA
Age (years), mean (SD)42.8 (11.9)60.9 (8.5)<0.00149.9 (12.9)64.1 (8.6)<0.001
Women84.6%77.9%0.00493.1%89.4%0.004
ANA positive93.8%93.4%0.7692.5%91.6%0.46
Scl70 positive63.2%60.0%0.2625.5%21.5%0.04
ACA positive5.5%6.6%0.4546.5%49.6%0.18
mRSS (years), mean (SD)18.7 (9.4)19.5 (10.4)0.188.1 (5.2)8.0 (5.2)0.66
Active disease43.8%52.7%0.00518.1%21.9%0.05
Elevated acute-phase reactants37.3%44.3%0.0221.8%26.3%0.03
Digital ulcers50.8%35.2%<0.00138.8%27.9%<0.001
Muscle weakness32.7%39.2%0.0221.0%22.5%0.43
Pulmonary fibrosis47.4%59.4%<0.00131.8%37.2%0.02
Lung restrictive defect47.9%50.3%0.2624.1%29.2%0.009
PAH17.7%26.3%<0.00116.8%23.4%<0.001
Dyspnoea37.8%52.2%<0.00131.3%37.0%0.008
Palpitations23.5%30.3%0.00620.6%23.6%0.07
Conduction block11.4%13.5%0.189.0%12.2%0.01
Diastolic dysfunction11.9%20.7%<0.00112.2%18.6%<0.001
Hypertension11.6%23.9%<0.00112.9%22.0%<0.001