Feature | Pre-PAH, n = 166 | Definite PH, n = 71 | |
---|---|---|---|
Female, n (%) | 138 (87) | 55 (87) | |
Ethnicity, n (%) | |||
White | 116 (75) | 42 (67) | |
African American | 25 (16) | 14 (22) | |
Hispanic | 9 (6) | 2 (3) | |
Asian | 3 (2) | 4 (6) | |
Age at SSc diagnosis, yrs, mean (SD) | 46 (13) | 45 (14) | |
Age at PHAROS enrollment, yrs, mean (SD) (range 35–79) | 57 (11) | 57 (10) | |
Time from first non-Raynaud’s until study entry, yrs, mean (SD) | 9 (8) | 9 (7) | |
Time from first Raynaud’s until study entry, yrs, mean (SD) | 13 (10) | 13 (10) | |
Limited SSc, n (%) | 58 (63) | 20 (57) | |
Autoantibodies, n (%) | n = 127 | n = 50 | |
Anticentromere | 28 (22) | 19 (38) | |
Antitopoisomerase | 23 (18) | 8 (16) | |
Isolated nucleolar ANA | 17 (13) | 19 (38) | |
BNP, pg/ml, mean (SD) | 35 (15) | 94 (70) | |
Entry criteria | |||
DLCO < 55% predicted | 91 (55) | 58 (79) | |
FVC/DLCO > 1.6 | 88 (53) | 51 (70) | |
Estimated RVSP on echo > 35 mm Hg | 50 (30) | 47 (64) | |
Cardiopulmonary | |||
Echo RVSP, mm Hg, mean (SD)* | 38 (10) | 57 (17) | |
Right heart catheterization, normal ranges31, mean (SD) | n = 16 | ||
mPAP (< 25 mm Hg)* | 20 (4) | 35 (11) | |
PVR (20–120 dyn×s/cm5)* | 180 (94) | 366 (230) | |
Cardiac output (4–8 l/min) | 5.4 (1.3) | 5.3 (1.5) | |
PCWP (6–12 mm Hg) | 8.2 (3.3) | 11.8 (4.1) | |
BORG Dyspnea Index, mean (SD)* | 1.9 (1.8) | 3.2 (2.3) | |
6MWD, m, mean (SD) | 393 (134) | 337 (135) | |
NYHA functional class, n (%)* | |||
Unclassified (no dyspnea) | 36 (23) | 5 (7) | |
1 | 51 (33) | 10 (15) | |
2 | 42 (27) | 29 (44) | |
3 | 25 (16) | 17 (25) | |
4 | 1 (1) | 6 (9) |
↵* Denotes statistically significant difference (p < 0.05). Autoantibody percentages calculated using the number of patients with an autoantibody present as the denominator (n = 112 for Pre-PAH, n = 50 for Definite PH). BNP: brain natriuretic peptide; FVC: % predicted forced vital capacity; DLCO: % predicted diffusing lung capacity for carbon monoxide; RVSP: estimated right ventricular systolic pressure on 2-dimensional Doppler echocardiography. Right heart catheterization (RHC) values with normal values in parentheses; mPAP: mean systolic 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. The Pre-PAH cohort includes 16 patients who underwent an RHC that revealed a normal mPAP.