Table 1 Prevalence of disease presentation among clinical scleroderma subsets.
| dcSSc | lcSSc | p (dcSSc vs lcSSC) | Other | Missing data (%) | |
|---|---|---|---|---|---|
| ACR criteria fulfilled | 100% | 100% | NA | 100% | 0 |
| Number of patients | 1349 (36.9%) | 2101 (57.5%) | <0.001 | 206 (5.6%) | 0 |
| Women | 81.1% | 90.9% | <0.001 | 86.9% | 0.4 |
| Age (years), mean (SD) | 52.3 (13.7) | 57.4 (13.1) | <0.001 | 52.7 (13.9) | 0.4 |
| Age at RO (years), mean (SD) | 42.9 (14.7) | 42.9 (14.5) | 0.98 | 40.6 (14.3) | 11.2 |
| Age at first non‐RO (years), mean (SD) | 44.8 (14.2) | 47.9 (13.4) | <0.001 | 43.8 (14.0) | 10.4 |
| Disease duration* (years), mean (SD) | 7.4 (6.9) | 9.6 (8.1) | <0.001 | 9.0 (7.5) | 10.7 |
| Time between RO and first non‐RO (years), mean (SD) | 1.9 (5.4) | 4.8 (8.5) | <0.001 | 3.2 (7.3) | 12.2 |
| ANA positive | 92.1% | 91.3% | 0.19 | 89.3% | 0.8 |
| Scl70 positive | 60.8% | 23.4% | <0.001 | 26.1% | 3.4 |
| ACA positive | 6.0% | 46.7% | <0.001 | 21.4% | 4.4 |
| mRSS, mean (SD) | 19.0 (10.0) | 8.1 (5.3) | <0.001 | 6.4 (6.6) | 3.0 |
| Active disease | 49.8% | 21.5% | <0.001 | 28.2% | 3.5 |
| Elevated acute phase reactants | 41.8% | 24.6% | <0.001 | 34.5% | 1.8 |
| Raynaud's phenomenon | 96.1% | 95.9% | 0.58 | 92.7% | 0.1 |
| Digital ulcers | 42.7% | 32.9% | <0.001 | 22.3% | 0.3 |
| Synovitis | 20.8% | 13.7% | <0.001 | 21.4% | 0.4 |
| Joint contractures (any joint) | 47.1% | 24.4% | <0.001 | 29.1% | 0.6 |
| Tendon friction rubs | 22.1% | 7.4% | <0.001 | 8.3% | 0.9 |
| Muscle weakness | 37.1% | 22.8% | <0.001 | 36.4% | 0.4 |
| Muscle atrophy | 21.1% | 10.8% | <0.001 | 20.9% | 1.1 |
| CK elevation | 11.3% | 4.4% | <0.001 | 12.1% | 2.8 |
| Oesophagus | 68.2% | 66.8% | 0.38 | 68.0% | 0.3 |
| Stomach | 26.6% | 22.8% | 0.04 | 21.8% | 0.7 |
| Intestine | 22.5% | 21.7% | 0.68 | 19.4% | 0.7 |
| Pulmonary fibrosis | 53.4% | 34.7% | <0.001 | 44.2% | 2.2 |
| Lung restrictive defect | 49.3% | 26.7% | <0.001 | 32.0% | 2.4 |
| % of predicted DLCO, mean (SD) | 64.0 (20.7) | 71.8 (21.0) | <0.001 | 71.6 (19.5) | 62.5 |
| PAH | 22.3% | 20.5% | 0.32 | 18.9% | 2.5 |
| PAH without fibrosis | 5.9% | 9.2% | <0.001 | 5.8% | 2.5 |
| PAH with fibrosis | 15.8% | 11.0% | <0.001 | 12.6% | 3.9 |
| Dyspnoea | 44.9% | 34.0% | <0.001 | 37.4% | 0.2 |
| Palpitations | 27.3% | 22.6% | 0.003 | 31.6% | 0.5 |
| Conduction block | 12.7% | 10.4% | 0.12 | 9.7% | 1.9 |
| Diastolic dysfunction | 16.6% | 15.4% | 0.42 | 15.0% | 2.3 |
| LVEF | 7.2% | 5.0% | 0.59 | 2.4% | 3.2 |
| Hypertension | 19.3% | 18.6% | 0.46 | 15.5% | 0.3 |
| Hypertensive renal crisis | 4.2% | 1.1% | <0.001 | 1.9% | 0.4 |
| Proteinuria | 9.2% | 3.7% | <0.001 | 10.2% | 1.5 |
ACA, anticentromere autoantibody; ACR, American College of Rheumatology; ANA, antinuclear antibodies; CK, creatine kinase; DLCO, diffusion capacity of the lung for carbon monoxide; dcSSc, diffuse cutaneous systemic sclerosis; lcSSc, limited cutaneous systemic scerosis; LVEF, left ventricular ejection fraction; mRSS, modified Rodnan Skin Score; NA, not applicable; PAH, pulmonary artery hypertension (assessed by echocardiography); RO, onset of Raynaud's phenomenon.
* Disease duration was calculated on the basis of the onset of the first non‐Raynaud's feature.