Patient characteristics
| Non-PH group (N=321) | PAH group (N=87) | |
|---|---|---|
| Demographics | ||
| Male, n/N (%) | 53/320 (16.6) | 22/86 (25.6) |
| Age (years), mean (SD) | 54.7 (11.8) | 61.1 (9.8) |
| Caucasian, n/N (%) | 258/319 (80.9) | 81/87 (93.1) |
| Body mass index (kg/m2), n | 317 | 86 |
| Mean (SD) | 25.2 (5.6) | 26.1 (5.5) |
| SSc characteristics | ||
| SSc duration* (months), n | 319 | 87 |
| Mean (SD) | 130.2 (96.1) | 163.0 (130.3) |
| SSc subtype, n/N (%) | ||
| Diffuse | 115/315 (36.5) | 18/86 (20.9) |
| Limited | 171/315 (54.3) | 61/86 (70.9) |
| Mixed/overlap | 29/315 (9.2) | 7/86 (8.1) |
| Current/past telangiectasias, n/N (%) | 218/321 (67.9) | 76/87 (87.4) |
| Functional capacity | ||
| 6-min walk distance (m), n | 243 | 66 |
| Mean (SD) | 412.5 (107.2) | 389.7 (106.6) |
| Borg dyspnoea index, n | 240 | 66 |
| Mean (SD) | 2.6 (1.8) | 3.1 (2.1) |
| WHO functional class, n | 306 | 87 |
| I/II/III/IV, n (%) | 133 (43.5)/123 (40.2)/50 (16.3)/0 | 16 (18.4)/40 (46.0)/30 (34.5)/1 (1.1) |
| Pulmonary function tests, n | 321 | 87 |
| DLCO % predicted | ||
| Mean (SD) | 48.0 (9.2) | 43.3 (10.5) |
| FVC % predicted/DLCO % predicted | ||
| Mean (SD) | 1.8 (0.5) | 2.2 (0.7) |
| Serum markers, n | 306 | 80 |
| NTproBNP (pg/ml) | ||
| Mean (SD) | 230.0 (538.6) | 516.4 (805.0) |
| Serum urate (mg/100 ml) | ||
| Mean (SD) | 4.7 (1.5) | 5.9 (1.5) |
| ACA positive, n/N (%) | 77/306 (25.2) | 40/80 (50.0) |
| Electrocardiography, n/N (%) | ||
| Right ventricular strain present | 7/291 (2.4) | 12/83 (14.5) |
| Right axis deviation† present | 10/291 (3.4) | 11/83 (13.3) |
| Echocardiography | ||
| Right atrium area (cm2), n | 286 | 82 |
| Mean (SD) | 13.4 (4.7) | 17.1 (6.2) |
| Right ventricle area (cm2), n | 291 | 82 |
| Mean (SD) | 15.0 (5.4) | 19.3 (6.8) |
| TR velocity (m/s), n | 255 | 78 |
| Mean (SD) | 2.4 (0.5) | 3.1 (0.7) |
| TR velocity (m/s), n/N (%) | ||
| ‘No TR’ ticked | 48/303 (15.8) | 6/84 (7.1) |
| ≤2.8 | 214/303 (70.6) | 30/84 (35.7) |
| >2.8 to ≤3.4 | 37/303 (12.2) | 23/84 (27.4) |
| >3.4 | 4/303 (1.3) | 25/84 (29.8) |
| Haemodynamics on right heart catheterisation | ||
| mPAP (mm Hg), n | 321 | 87 |
| Mean (SD) | 17.6 (3.8) | 32.5 (8.3) |
| PCWP (mm Hg), n | 318 | 87 |
| Mean (SD) | 8.5 (3.6) | 10.3 (3.2) |
| PVR (dyn·sec/cm5), n | 318 | 87 |
| Mean (SD) | 145.4 (64.6) | 370.6 (225.8) |
| Cardiac index (l/min/m2), n | 317 | 86 |
| Mean (SD) | 3.0 (0.7) | 2.9 (0.6) |
*From date of first non-Raynaud's symptom.
†QRS axis ≥90°.
ACA, anticentromere antibody; DLCO, pulmonary diffusing capacity for carbon monoxide; FVC, forced vital capacity; mPAP, mean pulmonary arterial pressure; NTproBNP, N-terminal probrain natriuretic peptide; PAH, pulmonary arterial hypertension; PH, pulmonary hypertension; PCWP, pulmonary capillary wedge pressure; PVR, pulmonary vascular resistance; SSc, systemic sclerosis; TR, tricuspid regurgitant jet.