Demographic and clinical characteristics of patients with systemic sclerosis (SSc) in the discovery and validation cohort
| Variable | Discovery cohort (n=79) | Validation cohort (n=83) |
| Females, n (%) | 72 (91.4%) | 65 (78.3%)* |
| Age, years | 56.6±13.7 | 58.5±13.9 |
| Raynaud duration, years | 15.8±11.2 | 12.5±9.9* |
| Disease duration, years | 11±9.5 | 8.8±9.1 |
| dcSSc, n (%) | 13 (16.5%) | 35 (50.7%)† |
| Autoantibodies, n (%) | ||
| ANA | 76 (96.2%) | 79 (95.8%) |
| ACA | 37 (46.8%) | 26 (31.3%)† |
| Anti-Topoisomerase-I | 27 (34.2%) | 41 (49.4%)† |
| ILD, n (%) | 27 (34.2%) | 44 (53%)† |
| FVC (% pred) | 100.3±20.1 (1) | 88.8±22.6† (2) |
| DLco (% pred) | 69.6±19.4 (1) | 55.1±17.4† (3) |
| PAH, n (%) | 3 (4.3%) | 4 (5.6%) |
| History of DU, n (%) | 36 (52.9%) (4) | 21 (25.3%)† |
| Telangectasia, n (%) | 48 (61.5%) (5) | 57 (68.7%) |
| Calcinosis, n (%) | 24 (30.1%) (5) | 13 (15.6%)† |
| Arthritis, n (%) | 10 (12.8%) (5) | 32 (38.6%)† |
| SRC, n (%) | 1 (1.4%) (5) | 0 (0%) |
| Myopathy, n (%) | 2 (2.6%) (5) | 1 (1.2%) |
| GORD, n (%) | 47 (60.3%) (5) | 52 (62.6%) |
| Intestinal_symptoms, n (%) | 43 (54.4%) | 31 (37.3%)* |
| Constipation | 22 (28.2%) (5) | 13 (15.6%) |
| Bloating | 35 (44.9%) (5) | 17 (20.5%) |
| Diarrhoea | 18 (23.1%) (5) | 15 (18.1%) |
| Abdominal pain | 10 (12.6%) | 13 (15.7%) |
| Weight loss, n (%) | 4 (5.1%) (5) | 14 (16.9%)* |
| Sicca syndrome, n (%) | 26 (32.9%) | 38 (46.3%) |
| Prednisone >5 mg/day, n (%) | 10 (12.6%) | 7 (8.4%) |
| Immunosuppressant, n (%) | 16 (20.2%) | 21 (25.3%) |
| MMF | 2 (2.5%) | 4 (4.8%) |
| MTX | 2 (2.5%) | 12 (14.5%) |
| LEF | 1 (1.4%) | 1 (1.2%) |
| AZA | 6 (7.6%) | 2 (2.4%) |
| CYC | 2 (2.5%) | 5 (6%) |
| Chronic iloprost, n (%) | 36 (45.6%) | 17 (20.5%)† |
| CCB, n (%) | 55 (69.6%) | 35 (42.2%)† |
| ASA, n (%) | 49 (62%) | 19 (23.9%)† |
| ACE-INH, n (%) | 8 (10.3%) | 15 (18.1%) |
| ERA, n (%) | 15 (19%) | 19 (22.9%) |
| PDE5, n (%) | 2 (2.5%) | 11 (13.3%) |
| Biologicals, n (%) | 9 (11.3%) | 0 (0%) |
| Abatacept | 2 (2.5%) | 0 (0%) |
| Tocilizumab | 5 (6.3%) | 0 (0%) |
| Anti-TNF | 2 (2.5%) | 0 (0%) |
| PGA | 28.2±15.5 | 46.6±20.2† |
Data from: (1) 64, (2) 82, (3) 79, (4) 54 and (5) 78 patients.
*p<0.05 versus discovery.
†p<0.01 versus discovery.
ACA, anticentromere antibodies; ACE-INH, ACE inhibitors; ANA, antinuclear antibodies; ASA, low-dose acetilsalycilc acid; AZA, azathioprine; CCB, calcium-channel blockers; CYC, cyclophosphamide; dcSSc, diffuse cutaneous SSc; DLco, diffusing capacity for carbon monoxide; DU, digital ulcers; ERA, endothelin receptor antagonists; FVC, forced vital capacity; GORD, gastro-oesophageal reflux disease confirmed by gastroscopy, manometry or X-rays; HCQ, hydroxycloroquine; ILD, interstitial lung disease; LEF, leflunomide; MMF, mycopheolate mophetil; MTX, methotrexate; PAH, pulmonary artery hypertension confirmed by right-heart catetherisation; PDE5, phosphodiesterase 5 inhibitors; PGA, physician's global assessment (0–100); SRC, scleroderma renal crisis; TNF, tumour necrosis factor.