| . | SSc-PAH patients (n = 50) . |
|---|---|
| Demographics and co-morbidities | |
| Female, n (%) | 48 (96) |
| Age [median (full range)], years | 65 (29–76) |
| Incident/prevalent PAH diagnosis, n (%) | 6/44 (12/88) |
| Intermediate-low/intermediate-high risk, n (%) | 30 (60)/20 (40) |
| Background haemodynamic and clinical measurements | |
| PVR (dynes/s/cm−5) | 291 (214–400) |
| mPAP (mmHg) | 31 (24–36) |
| FVC (%predicted) | 94.9 ± 18.9 |
| FEV1 (%predicted) | 86.7 ± 16.4 |
| DLCO (%predicted) | 40.6 ± 10.2 |
| Targeted PH therapies (n = 44, 88%) | |
| PDE5I, n (%) | 39 (78) |
| ERA, n (%) | 37 (74) |
| SGCS, n (%) | 2 (4) |
| Prostanoids (IV/oral IP receptor agonist), n (%) | 1 (2)/4 (8) |
| SSc details | |
| Limited/diffuse SSc, n (%) | 44/6 (88/12) |
| Overlap syndromes, n (%) | 4 (8) |
| Myositis, n (%) | 2 (4) |
| SLE, n (%) | 2 (4) |
| . | SSc-PAH patients (n = 50) . |
|---|---|
| Demographics and co-morbidities | |
| Female, n (%) | 48 (96) |
| Age [median (full range)], years | 65 (29–76) |
| Incident/prevalent PAH diagnosis, n (%) | 6/44 (12/88) |
| Intermediate-low/intermediate-high risk, n (%) | 30 (60)/20 (40) |
| Background haemodynamic and clinical measurements | |
| PVR (dynes/s/cm−5) | 291 (214–400) |
| mPAP (mmHg) | 31 (24–36) |
| FVC (%predicted) | 94.9 ± 18.9 |
| FEV1 (%predicted) | 86.7 ± 16.4 |
| DLCO (%predicted) | 40.6 ± 10.2 |
| Targeted PH therapies (n = 44, 88%) | |
| PDE5I, n (%) | 39 (78) |
| ERA, n (%) | 37 (74) |
| SGCS, n (%) | 2 (4) |
| Prostanoids (IV/oral IP receptor agonist), n (%) | 1 (2)/4 (8) |
| SSc details | |
| Limited/diffuse SSc, n (%) | 44/6 (88/12) |
| Overlap syndromes, n (%) | 4 (8) |
| Myositis, n (%) | 2 (4) |
| SLE, n (%) | 2 (4) |
Normally distributed data displayed as mean ± SD. Non-normally distributed data shown as median (interquartile range) unless otherwise specified.
DLCO, diffusion capacity of the lung for carbon monoxide; ERA, endothelin receptor antagonist; FEV1, forced expiratory volume in 1 s; FVC, forced vital capacity; IV, intravenous; mPAP, mean pulmonary arterial pressure; PAH, pulmonary arterial hypertension; PDE5I, phosphodiesterase type 5 inhibitor; PVR, pulmonary vascular resistance; SGCS, soluble guanylate cyclase stimulator; SLE, systemic lupus erythematosus; SSc, scleroderma/systemic sclerosis.
| . | SSc-PAH patients (n = 50) . |
|---|---|
| Demographics and co-morbidities | |
| Female, n (%) | 48 (96) |
| Age [median (full range)], years | 65 (29–76) |
| Incident/prevalent PAH diagnosis, n (%) | 6/44 (12/88) |
| Intermediate-low/intermediate-high risk, n (%) | 30 (60)/20 (40) |
| Background haemodynamic and clinical measurements | |
| PVR (dynes/s/cm−5) | 291 (214–400) |
| mPAP (mmHg) | 31 (24–36) |
| FVC (%predicted) | 94.9 ± 18.9 |
| FEV1 (%predicted) | 86.7 ± 16.4 |
| DLCO (%predicted) | 40.6 ± 10.2 |
| Targeted PH therapies (n = 44, 88%) | |
| PDE5I, n (%) | 39 (78) |
| ERA, n (%) | 37 (74) |
| SGCS, n (%) | 2 (4) |
| Prostanoids (IV/oral IP receptor agonist), n (%) | 1 (2)/4 (8) |
| SSc details | |
| Limited/diffuse SSc, n (%) | 44/6 (88/12) |
| Overlap syndromes, n (%) | 4 (8) |
| Myositis, n (%) | 2 (4) |
| SLE, n (%) | 2 (4) |
| . | SSc-PAH patients (n = 50) . |
|---|---|
| Demographics and co-morbidities | |
| Female, n (%) | 48 (96) |
| Age [median (full range)], years | 65 (29–76) |
| Incident/prevalent PAH diagnosis, n (%) | 6/44 (12/88) |
| Intermediate-low/intermediate-high risk, n (%) | 30 (60)/20 (40) |
| Background haemodynamic and clinical measurements | |
| PVR (dynes/s/cm−5) | 291 (214–400) |
| mPAP (mmHg) | 31 (24–36) |
| FVC (%predicted) | 94.9 ± 18.9 |
| FEV1 (%predicted) | 86.7 ± 16.4 |
| DLCO (%predicted) | 40.6 ± 10.2 |
| Targeted PH therapies (n = 44, 88%) | |
| PDE5I, n (%) | 39 (78) |
| ERA, n (%) | 37 (74) |
| SGCS, n (%) | 2 (4) |
| Prostanoids (IV/oral IP receptor agonist), n (%) | 1 (2)/4 (8) |
| SSc details | |
| Limited/diffuse SSc, n (%) | 44/6 (88/12) |
| Overlap syndromes, n (%) | 4 (8) |
| Myositis, n (%) | 2 (4) |
| SLE, n (%) | 2 (4) |
Normally distributed data displayed as mean ± SD. Non-normally distributed data shown as median (interquartile range) unless otherwise specified.
DLCO, diffusion capacity of the lung for carbon monoxide; ERA, endothelin receptor antagonist; FEV1, forced expiratory volume in 1 s; FVC, forced vital capacity; IV, intravenous; mPAP, mean pulmonary arterial pressure; PAH, pulmonary arterial hypertension; PDE5I, phosphodiesterase type 5 inhibitor; PVR, pulmonary vascular resistance; SGCS, soluble guanylate cyclase stimulator; SLE, systemic lupus erythematosus; SSc, scleroderma/systemic sclerosis.
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