Table 1.
Baseline characteristics of the study population
| SSc-PAH | SSc | Idiopathic PAH | Healthy control | |
|---|---|---|---|---|
| N | 18 | 21 | 15 | 14 |
| Men/woman (Nof patients) | 2/16 | 3/18 | 5/10 | 3/11 |
| Disease duration (years ± SD | 21.1 ± 7.6 | 19.1 ± 9.0 | 20.0 ± 10 | |
| Age (mean, years ± SD) | 69.8 ± 8.0 | 60.5 ± 9.3 | 62.5 ± 14.6 | 63.7 ± 10.1 |
| lcSSc/dcSSc (Nof patients) | 5/13 | 7/14 | ||
| ACA/Scl70 (Nof patients) | 5/13 | 7/14 | ||
| Digital ulcers (Nof patients) | 2 | 1 | ||
| sPAP eco | 73.1 ± 16.6 | 23.3 ± 4.4 | 68.7 ± 20.5 | |
| mPAP RHC | 43.0 ± 10.6 | 49.9 ± 21.0 | ||
| mRSS in dcSSc (mean ± SD) | 16.8 ± 2.4 | 10.6 ± 7.1 | ||
| mRSS in lcSSc (mean ± SD) | 3.6 ± 2.5 | 3.9 ± 2.3 | ||
| Prostanoid | Not | Not | Not | Not |
| Calcium-channel blockers | Not | Not | Not | Not |
| Phosphodiestererase-5 inhibitors | Not | Not | Not | Not |
| Endothelin-1 receptors antagonists | Not | Not | Not | Not |
| Vasoactive therapies | Not | Not | Not | Not |
ACA anti-centromere antibodies, dcSSc diffuse cutaneous SSc, lcSSc limited cutaneous SSc, mPAP RHC mean pulmonary artery pressure measured by RHC, mRSS modified Rodnan skin score, N number, PAH Pulmonary arterial hypertension, RHC right heart catheterization, SD standard deviation, sPAP eco systolic pulmonary artery pressure measured by echocardiography, SSc systemic sclerosis