PH Group | |||
---|---|---|---|
Characteristic | Group 1, PAH, n = 49 | Group 2, PVH, n = 7 | Group 3, PH-ILD, n = 15 |
Female, n (%) | 40 (91) | 5 (83) | 12 (80) |
Age at SSc diagnosis, yrs, mean (SD) | 44 (18) | 40 (21) | 44 (10) |
Age at PHAROS enrollment, yrs, mean (SD) | 59 (12) | 54 (13) | 54 (8) |
Race, n (% white)* | 31 (76) | 1 (20) | 8 (57) |
Limited SSc, n (%)* | 33 (72) | 3 (50) | 5 (36) |
Time from first non-Raynaud’s, yrs, mean (SD) | 10 (6) | 10 (7) | 9 (7) |
Time from first Raynaud’s, yrs, mean (SD) | 13 (9) | 15 (12) | 11 (9) |
RHC, mean (SD) | |||
mPAP (< 25 mm Hg) | 36 (12) | 35 (8) | 34 (9) |
PCWP (5–15 mm Hg)* | 11 (3) | 20 (4) | 11 (4) |
Cardiac output (3–4 l/min) | 5.2 (1.5) | 6.6 (0.6) | 5.4 (1.1) |
PVR (150–250 dyn×s/cm5) | 400 (240) | 297 (255) | 287 (115) |
Autoantibodies, n (%) | |||
Anticentromere | 15 (45) | 1 (25) | 1 (10) |
Scl-70* | 1 (3) | 0 | 6 (50) |
Nucleolar | 12 (40) | 2 (40) | 3 (30) |
Pulmonary function tests, mean % predicted (SD) | |||
FVC* | 86 (28) | 62 (26) | 56 (12) |
DLCO* | 43 (18) | 34 (7) | 30 (10) |
FVC/DLCO | 2.30 (1.1) | 2.06 (0.6) | 2.08 (0.6) |
Echocardiography | |||
RVSP (mm Hg), mean (SD) | 57 (21) | 45 (15) | 52 (16) |
6MWD, m, mean (SD) | 337 (133) | 420 (116) | 292 (132) |
NYHA functional class, n (%) | |||
Unclassified (no dyspnea) | 2 (4) | 2 (33) | 1 (8) |
1 | 9 (19) | 1 (17) | 0 |
2 | 20 (42) | 2 (33) | 7 (54) |
3 | 13 (27) | 1 (17) | 3 (23) |
4 | 4 (8) | 0 | 2 (15) |
↵* Denotes a statistically significant difference (p < 0.05). SSc: systemic sclerosis. SSc disease duration: time from onset of first non-Raynaud’s symptom to the date of first visit; FVC: % predicted forced vital capacity; DLCO: % predicted diffusing lung capacity for carbon monoxide; RVSP: estimated right ventricular pressure on 2-dimensional Doppler echocardiography. Right heart catheterization (RHC) values (n = 16) with normal values in parentheses; mPAP: mean pulmonary artery pressure; PVR: pulmonary vascular resistance; PCWP: pulmonary capillary wedge pressure. Functional class values; NYHA: New York Heart Association; 6MWD: 6-minute walk distance.