Characteristics of SSc-PAH patients from the discovery and the validation cohorts
| Discovery cohort | Validation cohort | |||
| N | N | |||
| Sex (female) | 15 | 14 (93%) | 24 | 17 (71%) |
| Age (years) | 15 | 65 (±7) | 24 | 68 (±9) |
| RP duration (years) | 10 | 19 (±13) | 8 | 18 (±12) |
| SSc duration (years) | 15 | 1.25(0.05 ;6) | 9 | 0.5(0 ;9) |
| BMI (kg/m2) | 15 | 28 (±4) | 16 | 28 (±6) |
| NYHA functional class | 15 | 18 | ||
| Class I | 0 (0%) | 0 (0%) | ||
| Class II | 7 (47%) | 6 (33%) | ||
| Class III | 8 (53%) | 10 (56%) | ||
| Class IV | 0 (0%) | 2 (11%) | ||
| BNP (pg/mL) | 12 | 147(69 ;505) | 14 | 84(39;264) |
| or Nt-pro-BNP (pg/mL), if BNP not available | / | / | 9 | 878(374;1124) |
| eGFR (mL/min) | 12 | 73 (±16) | 15 | 81 (±28) |
| ANA | 13 | 13 (100%) | 17 | 17 (100%) |
| ACA | 12 | 9 (75%) | 8 | 7 (88%) |
| ATA | 12 | 0 (0%) | 8 | 0 (0%) |
| Anti-U1RNP | 12 | 1 (8%) | 8 | 0 (0%) |
| LVEF (%) | 12 | 60 (±9) | 14 | 65 (±9) |
| FVC (% predicted) | 13 | 85 (±10) | 20 | 88 (±20) |
| TLC (% predicted) | 13 | 90 (±12) | 14 | 87 (±15) |
| DLCO (% predicted) | 15 | 35(8;52) | 10 | 41(33;65) |
| ILD on chest CT-scan | 15 | 6 (40%) | 24 | 5 (21%) |
| mPAP (mm Hg) | 15 | 44 (±10) | 24 | 37 (±8) |
| PAWP (mm Hg) | 15 | 10 (±2) | 24 | 10 (±3) |
| CO (L/min) | 15 | 4.9 (±1.6) | 24 | 4.6 (±1.2) |
| CI (L/min/m2) | 15 | 2.7 (±1.0) | 18 | 2.5 (±0.5) |
| PVR (WU) | 15 | 8.5 (±5.0) | 24 | 6.2 (±2.3) |
ACA, anticentromere antibodies; ANA, antinuclear antibodies; Anti-U1RNP, anti-U1-ribonucleoprotein antibodies; ATA, anti-topoisomerase I antibodies; BMI, body mass index; BNP, brain natriuretic peptide; CI, Cardiac Index; CO, cardiac output; DLCO, diffusing capacity of the lung for carbon monoxide; eGFR, estimated glomerular filtration rate; FVC, forced vital capacity; ILD, interstitial lung disease; LVEF, left ventricular ejection fraction; mPAP, mean pulmonary arterial pressure; Nt-pro-BNP, N-terminal pro-BNP; NYHA, New York Heart Association; PAH, pulmonary arterial hypertension; PAWP, pulmonary arterial wedge pressure; PVR, pulmonary vascular resistance; RP, Raynaud phenomenon; SSc, systemic sclerosis; TLC, total lung capacity; WU, wood unit.