Table 1.
Demographics and Clinical Features of Patients with SSc, by Presence of PH.
| Variable | SSc Only | SSc-PH |
|---|---|---|
| (N = 64) | (N = 66) | |
|
| ||
| Demographics | ||
| Age, years (mean ± SD) | 53.4 ± 10.8 | 57.7 ± 11.0 |
| Male Sex, n (%) | 7 (10.9%) | 17 (25.8%) |
| Diffuse Cutaneous SSc, n (%) | 29 (45.3%) | 29 (43.9%) |
| Former or Current Smoker, n (%) | 33 (51.6%) | 36 (54.5%) |
| PH Classification | ||
| Pre-capillary PH, n (%) | 0 | 34 (51.5%) |
| Post-capillary PH, n (%) | 0 | 10 (15.2%) |
| Combined pre- and post-capillary PH, n (%) | 0 | 4 (6.1%) |
| BNP Measurements # | ||
| BNP < 100 pg/mL | 28 (82.4%) | 33 (51.6%) |
| BNP ≥ 100 pg/mL and < 400 pg/mL | 6 (17.6%) | 19 (29.7%) |
| BNP ≥ 400 pg/mL | 0 | 12 (18.8%) |
| Pulmonary Function Testing | ||
| FVC, % predicted (mean ± SD) | 79.9 ± 19.2 | 69.8 ± 17.6 |
| DLCO, % predicted (mean ± SD) | 58.9 ± 18.4 | 44.3 ± 16.0 |
| FVC/DLCO Ratio (mean ± SD) | 1.5 ± 0.4 | 1.7 ± 0.7 |
| Electrocardiography | ||
| Left Axis Deviation, n (%) | 4 (6.3%) | 12 (18.2%) |
| Right Axis Deviation, n (%) | 0 | 4 (6.1%) |
| Right Bundle Branch Block, n (%) | 4 (6.3%) | 5 (7.6%) |
| Echocardiography | ||
| Aortic Regurgitation, n (%) | 2 (3.1%) | 11 (16.7%) |
| Mitral Regurgitation, n (%) | 15 (23.4%) | 20 (30.3%) |
| Tricuspid Regurgitation, n (%) | 26 (40.6%) | 37 (56.1%) |
| Pulmonic Regurgitation, n (%) | 5 (7.8%) | 15 (22.7%) |
| LA Dilation, n (%) | 9 (14.1%) | 25 (37.9%) |
| LVEF, % (mean ± SD) | 62.2 ± 4.8 | 60.7 ± 9.5 |
| LV Diastolic Dysfunction, n (%) | 6 (9.4%) | 21 (31.8%) |
| LV Hypertrophy, n (%) | 8 (12.5%) | 19 (28.8%) |
| RA Dilation, n (%) | 1 (1.6%) | 15 (22.7%) |
| RV Dilation, n (%) | 2 (3.1%) | 24 (36.4%) |
| RV Dysfunction, n (%) | 0 | 10 (15.2%) |
| Pericardial Effusion, n (%) | 16 (25.0%) | 21 (31.8%) |
| Insufficient TR Jet for ePASP, n (%) | 21 (32.8%) | 14 (21.9%) |
| ePASP > 35 mmHg, n (%) | 6 (9.4%) | 38 (57.6%) |
| ePASP > 40 mmHg, n (%) | 2 (3.1%) | 30 (45.5%) |
| ePASP, mmHg (mean ± SD)* | 28.6 ± 6.9 | 48.3 ± 16.2 |
| Chest CT Findings | ||
| PA Diameter, mm (mean ± SD) | 26.3 ± 3.6 | 31.1 ± 5.5 |
Abbreviations: BNP: B-type natriuretic peptide; CT: computed tomography; DLCO: Diffusing capacity of carbon monoxide; ePASP: Estimated pulmonary artery systolic pressure; FVC: Forced vital capacity; LA: Left atrial; LV: Left ventricular; LVEF: Left ventricular ejection fraction; PA: Pulmonary artery; PH: Pulmonary hypertension; RA: Right atrial; RV: Right ventricular; SSc: Systemic sclerosis; TR: Tricuspid regurgitant
#
For BNP, SSc Only: N = 34; SSc-PH: N = 64.
*
For ePASP, SSc Only: N = 43; SSc-PH: N = 51.