Patient characteristics of pulmonary arterial hypertension (PAH) and non-pulmonary hypertension (non-PH) systemic sclerosis (SSc) patients in the Sheffield Confirmatory Cohort
| Non-PH | PAH | All | |
| All patients | 22 | 22 | 44 |
| Demographic and clinical parameters | |||
| Age years | |||
| Patients | 22 | 22 | 44 |
| Mean±sd | 62.3±9.7 | 68.1±8.0 | 65.2±9.2 |
| Median (IQR) | 61.5 (56.3–67.8) | 69.0 (63.3–72.0) | 64.5 (58.8–72.0) |
| Minimum–maximum | 45–84 | 53–84 | 45–84 |
| p=0.026 | |||
| Sex | |||
| Patients | 22 | 22 | 44 |
| Female | 20 (90.9) | 15 (68.2) | 35 (79.5) |
| Male | 2 (9.1) | 7 (31.8) | 9 (20.5) |
| p=0.091 | |||
| BMI kg·m−2 | |||
| Patients | 22 | 20 | 42 |
| Mean±sd | 27.7±5.8 | 27.5±6.9 | 27.6±6.3 |
| Median (IQR) | 28.1 (23.9–32.6) | 28.1 (21.7–31.7) | 28.1 (21.9–32.0) |
| p=0.782 | |||
| Smoking history | 8/19 (42.1) | 13/19 (68.4) | 21/38 (55.3) |
| p=1 | |||
| SSc subtype | |||
| Patients | 22 | 22 | 44 |
| Limited | 21 (95.5) | 22 (100) | 43 (97.7) |
| Diffuse | 1 (4.5) | 0 (0) | 1 (2.3) |
| Mixed | 0 (0) | 0 (0) | 0 (0) |
| p=1 | |||
| Echocardiography/CTPA/cardiac MRI | |||
| Qualitative evaluation of right ventricle pump | |||
| Patients | 22 | 22 | 44 |
| Normal | 18 (81.8) | 11 (50.0) | 29 (65.9) |
| Mild impairment | 4 (18.2) | 2 (9.1) | 6 (13.6) |
| Moderate impairment | 0 (0) | 1 (4.5) | 1 (2.3) |
| Severe impairment | 0 (0) | 8 (36.4) | 8 (18.2) |
| p=0.0083 | |||
| Right atrium area (maximum) cm2 | |||
| Patients | 22 | 22 | 44 |
| Mean±sd | 16.0±5.4 | 22.5±9.6 | 19.2±8.4 |
| Median (IQR) | 15.4 (11.5–17.1) | 19.8 (13.7–29.9) | 16.9 (13.2–23.7) |
| p=0.018 | |||
| TRV m·s−1 | |||
| Patients | 17 | 22 | 39 |
| Mean±sd | 2.7±0.3 | 3.7±0.8 | 3.2±0.8 |
| Median (IQR) | 2.7 (2.5–2.8) | 3.4 (3.1–4.2) | 3.1 (2.7–3.5) |
| p=1.98e-05 | |||
| Electrocardiography | |||
| Presence of right axis deviation | 1/20 (5.0) | 9/22 (40.9) | 10/42 (23.8) |
| p=0.45 | |||
| Right heart catheterisation | |||
| PVR dyn·s·cm−5 | |||
| Patients | 22 | 22 | 44 |
| Mean±sd | 146.4±56.5 | 548.2±375.4 | 347.3±334.2 |
| Median (IQR) | 139.5 (116.8–170.8) | 396.0 (271.2–850.2) | 219.0 (142.8–395.5) |
| p=1.5e-09 | |||
| mPAP mmHg | |||
| Patients | 22 | 22 | 44 |
| Mean±sd | 20.7±3.1 | 41.7±15.3 | 31.2±15.2 |
| Median (IQR) | 21.0 (19.0–22.0) | 38.0 (30.0–52.0) | 26.0 (21.0–37.0) |
| p=3.0e-08 | |||
| PAWP mmHg | |||
| Patients | 22 | 22 | 44 |
| Mean±sd | 9.1±3.8 | 11.7±4.9 | 10.4±4.5 |
| Median (IQR) | 8.5 (7.0–11.8) | 12.0 (8.0–14.0) | 10.0 (7.8–13.2) |
| p=0.071 | |||
| log10(NT-proBNP) pg·mL−1 | |||
| Patients | 22 | 22 | 44 |
| Mean±sd | 2.6±0.5 | 3.3±0.5 | 3.0±0.6 |
| Median (IQR) | 2.7 (2.4–2.9) | 3.3 (3.0–3.7) | 2.9 (2.6–3.3) |
| p=9.3e-05 | |||
| Pulmonary function tests | |||
| FVC % pred | |||
| Patients | 22 | 22 | 44 |
| Mean±sd | 90.8±22.6 | 101.2±15.7 | 96.0±20.0 |
| Median (IQR) | 94.7 (73.4–107.9) | 100.8 (90.9–111.5) | 96.3 (80.9–110.9) |
| p=0.13 | |||
| DLCO % pred | |||
| Patients | 21 | 22 | 43 |
| Mean±sd | 56.2±13.4 | 43.9±12.7 | 49.9±14.3 |
| Median (IQR) | 55.1 (46.7–67.3) | 43.6 (39.6–47.0) | 46.7 (42.0–56.5) |
| p=0.002 |
Data are presented as n, n (%) or n/N (%), unless otherwise stated. IQR: interquartile range; BMI: body mass index; CTPA: computed tomography pulmonary angiography; MRI: magnetic resonance imaging; TRV: tricuspid regurgitant velocity; PVR: pulmonary vascular resistance; mPAP: mean pulmonary arterial pressure; PAWP: pulmonary arterial wedge pressure; NT-proBNP: N-terminal pro-brain natriuretic peptide; FVC: forced vital capacity; DLCO: diffusing capacity of the lung for carbon monoxide. Comparison of PAH and non-PH groups performed using the Wilcoxon rank-sum test for continuous variables (and for the “qualitative evaluation of right ventricle pump” after conversion of the ordered factor levels to 1–4 values) and Fisher's exact test for categorical variables.