Table 1

Baseline demographics and clinical characteristics of patients with idiopathic PAH (IPAH) or systemic sclerosis-associated PAH (SSc-APAH)*

CharacteristicIPAH (n=279)SSc-APAH (n=228)p Value
Age (years)56 (43–67)58 (51–67)0.02
Female214 (76.7)203 (89.0)0.0003
Race
 Caucasian209 (77.1)165 (72.4)0.53
 Black34 (12.6)39 (17.1)
 Hispanic20 (7.4)18 (7.9)
 Others8 (2.9)6 (2.6)
Raynaud's phenomenon11/212 (5.2)106/189 (56.1)<0.0001
Peripheral oedema81/214 (37.9)78/169 (46.2)0.10
Body mass index (kg/m2)27.8 (23.9–33.3)25.7 (22.6–32.4)0.01
Systolic BP (mm Hg)119 (104–130)116 (102–130)0.13
Diastolic BP (mm Hg)70 (62–79)69 (60–78)0.65
Heart rate (bpm)80 (72–90)82 (74–93)0.09
Age at PAH diagnosis (years)53 (40–64)57 (49–65)0.0008
Increased P2117/248 (47.2)95/195 (48.7)0.75
Right ventricular gallop21/213 (9.9)13/159 (8.2)0.58
Functional class
 I25 (9.3)11 (5.2)0.08
 II116 (43.3)79 (37.3)
 III110 (41.0)110 (51.9)
 IV17 (6.3)12 (5.7)
  • * Numbers are absolute frequencies and proportions unless otherwise stated.

  • Denominator unless otherwise stated.

  • Median (25th, 75th percentiles).

  • BP, blood pressure; P2, pulmonic closure sound; PAH, pulmonary arterial hypertension.