Table 2.

Demographic, clinical, and laboratory features of patients with Pre-PAH and Definite PH.

FeaturePre-PAH, n = 166Definite PH, n = 71
Female, n (%)138 (87)55 (87)
Ethnicity, n (%)
  White116 (75)42 (67)
  African American25 (16)14 (22)
  Hispanic9 (6)2 (3)
  Asian3 (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 = 127n = 50
  Anticentromere28 (22)19 (38)
  Antitopoisomerase23 (18)8 (16)
  Isolated nucleolar ANA17 (13)19 (38)
BNP, pg/ml, mean (SD)35 (15)94 (70)
Entry criteria
  DLCO < 55% predicted91 (55)58 (79)
  FVC/DLCO > 1.688 (53)51 (70)
  Estimated RVSP on echo > 35 mm Hg50 (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)
    151 (33)10 (15)
    242 (27)29 (44)
    325 (16)17 (25)
    41 (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.