Table 4.

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

PH Group
CharacteristicGroup 1, PAH, n = 49Group 2, PVH, n = 7Group 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 (%)
  Anticentromere15 (45)1 (25)1 (10)
  Scl-70*1 (3)06 (50)
  Nucleolar12 (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/DLCO2.30 (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)
  19 (19)1 (17)0
  220 (42)2 (33)7 (54)
  313 (27)1 (17)3 (23)
  44 (8)02 (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.