Epidemiological and clinical features of the study population (n = 50).
| Feature | |
|---|---|
| Female/male | 43/7 |
| Age, mean ± SD yrs | 51 ± 14.5 |
| Age at onset of disease, mean ± SD yrs | 40 ± 17 |
| Raynaud’s phenomenon duration, mean ± SD yrs | 15 ± 10 |
| Subset* dsSSc/lcSSc, n | 27/23 |
| ANA pattern, n (%) | |
| Speckled | 9 (18) |
| Speckled and nucleolar | 18 (36) |
| Nucleolar | 4 (8) |
| Centromere | 19 (38) |
| SSc-specific autoantibody, n (%) | |
| Anti-topo I | 23 (46) |
| ACA | 19 (38) |
| None | 8 (16) |
| DAI** | 1.8 ± 0.6 |
| DSI† | 4.7 ± 2.2 |
| Disease features, n (%) | |
| Interstitial lung disease | 23 (46) |
| Scleroderma renal crisis | 3 (6) |
| Myositis | 4 (8) |
| Raynaud’s phenomenon | 50 (100) |
| Gastroesophageal reflux disease | 45 (90) |
| Digital ulcer | 22 (44) |
| Pulmonary hypertension | 18 (36) |
| Sicca syndrome | 6 (12) |
| Autoimmune thyroiditis | 8 (16) |
| Primary biliary cirrhosis | 4 (8) |
| Previous or current iron deficiency anemia | 7 (14) |
| IgA deficit | 1 (2) |
| Positive family history for celiac disease, n (%) | 1 (2) |
dcSSc: diffuse cutaneous SSc: lcSSc: limited cutaneous SSc: ANA: antinuclear antibody; anti-topo I: anti-topoisomerase I, ACA: anticentromere antibody.
↵* According to Le Roy, et al1;
↵** Disease Activity Index according to Valentini, et al30;
↵† Disease Severity Index according to Medsger, et al31.