Table 4 Prevalence of disease presentation according to autoantibody serology.
| ANA positive | Scl70 positive | ACA positive | p (Scl70 vs ACA) | |
|---|---|---|---|---|
| Number of patients | 3346 | 1330 | 1106 | <0.001 |
| Presenting as dcSSC | 37.1% | 60.0% | 7.3% | <0.001 |
| Presenting as lcSSC | 57.4% | 36.1% | 88.7% | <0.001 |
| Presenting as “other” | 5.5% | 3.9% | 4.0% | 0.88 |
| Women | 87.3% | 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 disease | 32.7% | 45.2% | 18.9% | <0.001 |
| Elevated acute‐phase reactants | 31.9% | 42.6% | 20.7% | <0.001 |
| Raynaud's phenomenon | 96.3% | 97.4% | 96.7% | 0.45 |
| Digital ulcers | 36.7% | 44.8% | 31.2% | <0.001 |
| Synovitis | 16.7% | 21.4% | 11.9% | <0.001 |
| Joint contractures (any joint) | 33.7% | 44.5% | 17.6% | <0.001 |
| Tendon friction rubs | 13.1% | 18.9% | 6.0% | <0.001 |
| Muscle weakness | 28.4% | 32.2% | 22.7% | <0.001 |
| Muscle atrophy | 14.6% | 16.1% | 9.5% | <0.001 |
| CK elevation | 7.6% | 8.7% | 2.9% | <0.001 |
| Oesophagus | 67.9% | 68.0% | 70.7% | 0.18 |
| Stomach | 24.5% | 24.1% | 26.9% | 0.11 |
| Intestine | 22.5% | 20.7% | 25.1% | 0.01 |
| Pulmonary fibrosis | 42.6% | 60.2% | 21.3% | <0.001 |
| Lung restrictive defect | 35.8% | 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 |
| PAH | 21.1% | 23.2% | 22.0% | 0.36 |
| PAH without fibrosis | 8.0% | 5.0% | 13.0% | <0.001 |
| PAH with fibrosis | 12.7% | 17.2% | 8.0% | <0.001 |
| Dyspnoea | 38.6% | 44.5% | 29.4% | <0.001 |
| Palpitations | 24.8% | 27.2% | 23.2% | 0.01 |
| Conduction block | 11.2% | 13.6% | 9.1% | <0.001 |
| Diastolic dysfunction | 15.7% | 17.7% | 12.7% | 0.001 |
| Reduced LVEF | 5.7% | 5.9% | 5.2% | 0.29 |
| Hypertension | 18.5% | 14.4% | 20.0% | <0.001 |
| Hypertensive renal crisis | 2.3% | 2.0% | 1.3% | 0.15 |
| Proteinuria | 6.0% | 7.8% | 2.7% | <0.001 |
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.