Table 1.
Cohort characteristics (n = 1303)
| Characteristic | Values |
|---|---|
| Female | 1125 (86.3%) |
| Race | |
| Caucasian | 1178 (90.4%) |
| Asian | 59 (4.5%) |
| Aboriginal-Islander | 15 (1.2%) |
| Other | 18 (1.4%) |
| Disease subtype | |
| Limited | 974 (74.8%) |
| Diffuse | 329 (25.2%) |
| Age at scleroderma onset, years | 46.40 ± 14.26 |
| Age at recruitment, years | 57.71 ± 12.54 |
| Disease duration at recruitment, years | 11.26 ± 10.31 |
| Duration of follow-up, years | 3.46 ± 2.69 |
| Ever smoked | 639 (49.0%) |
| Autoantibodies | |
| ANCA | 116 (8.9%) |
| Anti-Scl-70 | 181 (13.9%) |
| Anti-centromere | 613 (47.0%) |
| Antinuclear antibody | 1222 (93.8%) |
| Rheumatoid factor | 337 (25.9%) |
| Anti-Ro | 87 (6.7%) |
| Anti-La | 20 (1.5%) |
| Anti-RNP | 26 (2.0%) |
| Anti-dsDNA | 42 (3.2%) |
| Anti-Sm | 8 (0.6%) |
| Anti-Jo-1 | 7 (0.5%) |
| Anti-Scl/PM | 15 (1.2%) |
| Anti-cardiolipin | 262 (20.1%) |
| Anti-beta 2 glycoprotein | 94 (7.2%) |
| Lupus anti-coagulant | 35 (2.7%) |
| Anti-RNA polymerase | 116 (8.9%) |
| Clinical characteristics and complications | |
| Pulmonary arterial hypertension | 163 (12.5%) |
| Interstitial lung disease | 311 (23.9%) |
| Renal crisis | 35 (2.7%) |
| Digital ulcers | 646 (49.6%) |
| SIBO | 37 (2.8%) |
| GAVE | 112 (8.6%) |
| Oesophageal dysmotility | 132 (10.1%) |
| Oesphageal stricture | 146 (11.2%) |
| Synovitis | 517 (39.7%) |
| Malignancy | 252 (19.3%) |
| Hospitalisation | 490 (37.6%) |
| Deep vein thrombosis (DVT) | 91 (7.0%) |
| Pulmonary embolus (PE) | 46 (3.5%) |
| Overlap features with another connective tissue disease | 76 (5.8%) |
| Overlap syndrome with rheumatoid arthritis | 27 (2.1%) |
| Overlap syndrome with polymyositis | 13 (1.0%) |
| Overlap syndrome with Sjogren’s | 24 (1.8%) |
| Overlap syndrome with SLE | 12 (0.9%) |
| Overlap syndrome with dermatomyositis | 1 (0.1%) |
| Treatments | |
| Prednisolone | 580 (44.5%) |
| Azathioprine | 105 (8.1%) |
| Mycophenolate | 103 (7.9%) |
| Cyclophosphamide | 118 (9.1%) |
| Calcium channel antagonist | 847 (65.0%) |
| Topical vasodilator | 105 (8.1%) |
| Iloprost | 161 (12.4%) |
| Penicillamine | 108 (8.3%) |
| Rituximab | 11 (0.8%) |
| Abatacept | 1 (0.1%) |
| TNF alpha inhibitor | 11 (0.8%) |
| Tocilizumab | 5 (0.4%) |
| Biologics | 26 (2.0%) |
Values are given as number (%), mean ± SD or median (IQR)
Abbreviations: ANA anti nuclear antibodies, ANCA anti-neutrophil cytoplasmic antibodies, anti-phospholipid antibodies: any one or combination of anti-cardiolipin, anti-beta-2-glycoprotein and lupus anticoagulant, anti-dsDNA anti-double stranded deoxyribonucleic acid, anti-RNP ribonucleoprotein, SIBO small intestinal bacterial overgrowth, SLE systemic lupus erythematosus, anti-Sm anti-Smith