Baseline characteristics of the 85 incident SSc-PAH patients enrolled in the French PAH registry between 2006 and 2009
| Parameter | N | Value |
|---|---|---|
| Demographics | ||
| Female gender | 85 | 70 (82) |
| Age, years | 85 | 64.9±12.2 |
| Ever/never smoker | 85 | 30 (35)/55 (65) |
| Disease characteristics | ||
| Limited/diffuse cutaneous SSc | 85 | 74 (87)/11 (13) |
| Antinuclear antibodies | 83 | 54 (65) |
| ACA positive | 83 | 33 (40) |
| ATA positive | 83 | 3 (4) |
| Functional capacity | ||
| NYHA FC, I/II/III/IV | 82 | 0 (0)/17 (21)/55 (67)/10 (12) |
| 6MWD, m | 75 | 259±126 |
| Echocardiography | ||
| Pericardial effusion present | 55 | 14 (25) |
| Pulmonary function tests | ||
| FVC, % predicted | 73 | 92±21 |
| TLC, % predicted | 73 | 94±15 |
| FEV1, % predicted | 72 | 90±22 |
| KCO, % predicted | 64 | 53±20 |
| Blood gases | ||
| PaO2, mm Hg | 57 | 70±18 |
| PaCO2, mm Hg | 54 | 33±9 |
| SaO2, % | ||
| Before 6MWT | 64 | 95±3 |
| Post 6MWT | 60 | 88±5 |
| Blood analysis | ||
| BNP, ng/l | 48 | 463±573 |
| Haemodynamics | ||
| RAP, mm Hg | 84 | 7±5 |
| mPAP, mm Hg | 85 | 41±11 |
| PCWP, mm Hg | 85 | 8±4 |
| Cardiac output, l/min | 85 | 4.44±1.56 |
| Cardiac index, l/min/m2 | 83 | 2.64±0.78 |
| PVR, dyn/sec/cm−5 | 85 | 680±355 |
| SV, ml | 71 | 57±26 |
| SV/PP, ml/mm Hg | 70 | 1.81±1.59 |
| RVSWI, g.m/m2 | 68 | 14.81±5.22 |
| Heart rate, bpm | 71 | 83±15 |
Data are mean±SD or n (%).
6MWD/T, six-min walking distance/test; ACA, anticentromere antibody; ATA, antitopoisomerase I antibody; BNP, brain natriuretic peptide; FVC, forced vital capacity; FEV1, forced expiratory volume in 1 s; KCO, diffusing capacity for carbon monoxide corrected for alveolar volume; mPAP, mean pulmonary arterial pressure; NYHA FC, New York Heart Association functional class; PAH, pulmonary arterial hypertension; PaO2/PaCO2 partial pressure of oxygen/carbon dioxide in arterial blood; PCWP, pulmonary capillary wedge pressure; PVR, pulmonary vascular resistance; RAP, right atrial pressure; RVSWI, right ventricle stroke work index; SaO2, oxygen saturation measured by pulse oximetry; SSc, systemic sclerosis; SV, stroke volume; SV/PP, pulmonary artery capacitance; TLC, total lung capacity.