Patient characteristics
| Characteristics | Patients (n=168) |
| Age, years±SD | 57.8±15 |
| Female, n (%) | 147 (87.5) |
| Smoking, n (%) | |
| Current or past | 20 (12) |
| Never | 136 (81) |
| NA | 12 (7) |
| SSc type, n (%) | |
| Limited | 69 (41) |
| Diffuse | 99 (59) |
| Disease duration, years±SD* | 8.2±13.5 |
| Modified Rodnan skin score (0–51), mean (Q1, Q3) | 8.0 (4.0, 19.0) |
| ADU, n (%) | 48 (29) |
| Digital tip ulcerations, n (%) | 44 (26) |
| Digital gangrene, n (%) | 4 (3) |
| Raynaud’s severity score (0–4), mean (Q1, Q3) | 1.0 (1.0, 3.0) |
| PAH | |
| Suspected PAH on echocardiography, n (%) | 19 (11) |
| sPAP (mm Hg), mean±SD | 31.8±13.7 |
| PAH on RHC, n (%) | 12 (7) |
| mPAP (mm Hg), mean±SD | 30.9±15 |
| LVEF on echocardiography (%), mean±SD | 66±9 |
| ILD, n (%) | 73 (43) |
| FVC, % predicted ±SD | 94.6±24.7 |
| DLCO, % predicted ±SD | 69.1±21.1 |
| SSc-related autoantibodies, n (%) | |
| Anticentromere, n (%) | 76 (45) |
| Anti-Scl70, n (%) | 60 (36) |
| LA, n (%) | 17 (10) |
| Overall | High (>40 U/mL) | |
| aCL, n (%) | ||
| Any isotype (global) | 14 (8) | 6 (4) |
| IgG | 11 (6) | 6 (4) |
| IgM | 3 (2) | 0 (0) |
| Anti-β2GP1, n (%) | ||
| Any isotype | 14 (8) | 10 (6) |
| IgG | 8 (5) | 6 (4) |
| IgM | 8 (5) | 4 (3) |
| ≥1 aPL, n (%) | 31 (18) | 26 (15) |
| Triple positivity, n (%) | 3 (2) | 1 (1) |
| APS, n (%) | 7 (4) | |
| Related-arterial thrombosis, n (%) | 4 (2) | |
| Related-venous thrombosis, n (%) | 3 (2) | |
| Obstetrical manifestation, n (%) | 0 (0) | |
| Overall arterial thrombosis history, n (%) | 5 (3) | |
| Overall venous thrombosis history, n (%) | 13 (8) | |
*Time from first non-Raynaud’s symptom.
aCL, anticardiolipin; ADU, active digital ulceration; aPL, antiphospholipid antibodies; APS, antiphospholipid syndrome; DLCO, diffusing capacity of the lung for carbon monoxide; FVC, forced vital capacity; β2GP1, beta-2 glycoprotein 1;Ig, immunoglobulin; ILD, interstitial lung disease; LA, lupus anticoagulant; LVEF, left ventricular ejection fraction; mPAP, mean pulmonary arterial pressure;NA, not available; PAH, pulmonary arterial hypertension; Q1/Q3, quartile 1/quartile 3; RHC, right heart catheterisation; sPAP, systolic pulmonary arterial pressure; SSc, systemic sclerosis.