Table 1

Baseline demographics and clinical characteristics

PAH-CTDPAH-SSc
Combination therapy
(n=117)
Monotherapy (pooled)
(n=99)
Combination therapy
(n=81)
Monotherapy (pooled)
(n=56)
Mean age (SD) (years)59.1 (12.1)58.3 (13.0)62.6 (8.9)60.8 (10.3)
Female, n (%)101 (86)88 (89)67 (83)49 (88)
WHO-FC, n (%)
 II33 (28)30 (30)23 (28)17 (30)
 III84 (72)69 (70)58 (72)39 (70)
6MWD (m)
 Mean (SD)326 (89)330 (96)319 (91)336 (95)
 Median334347330355
Haemodynamic criteria
 RAP (mm Hg), mean (SD)*7.4 (4.2)7.7 (4.4)7.2 (4.1)7.6 (4.5)
 CI (L/min/m2), mean (SD)†2.6 (0.6)2.7 (0.7)2.6 (0.6)2.6 (0.6)
 PAP (mm Hg), mean (SD)42.6 (10.6)43.7 (10.6)42.3 (10.7)43.4 (10.8)
 PAWP (mm Hg), mean (SD)‡8.5 (3.2)9.6 (3.4)8.4 (3.0)9.2 (3.3)
 PVR (dyne s/cm5), mean (SD)638 (282)627 (277)639 (279)633 (291)
 Median (Q1–Q3) NT-proBNP (ng/L)§978 (309–2757)1047 (337–2334)1261 (265–3144)871 (332–2312)
Prior medications, n (%)
 Immunosuppressants17 (14.5)15 (15.2)6 (7.4)6 (10.7)
 Oral corticosteroids22 (18.8)21 (21.2)12 (14.8)9 (16.1)
 Median days from diagnosis to treatment23252319.5
 Mean % predicted TLC (SD)¶88.3 (15.83)88.1 (19.52)88.9 (16.82)90.2 (21.65)
 Mean % predicted FEV1 (SD)85.1 (19.27)81.2 (16.32)87.3 (20.38)85.8 (17.38)
  • *Data not available in some patients (PAH-CTD: combination therapy, n=115).

  • †Data not available in some patients (PAH-CTD: monotherapy, n=98; SSc-PAH: monotherapy, n=55).

  • ‡Data not available in some patients (PAH-CTD: combination therapy, n=115; monotherapy, n=97; SSc-PAH: combination therapy, n=80; monotherapy, n=54).

  • §Data not available in some patients (PAH-CTD: combination therapy, n=111; monotherapy, n=97; SSc-PAH: combination therapy, n=77; monotherapy, n=55).

  • ¶Data not available in some patients (PAH-CTD: combination therapy, n=116; monotherapy, n=98; SSc-PAH: combination therapy, n=80; monotherapy, n=56).

  • CI, cardiac index; CTD, connective tissue disease; FEV1, forced expiratory volume in one second; 6MWD, 6 min walking distance; NT-proBNP, N-terminal pro-B-type natriuretic peptide; PAH, pulmonary arterial hypertension; PAP, pulmonary arterial pressure; PAWP, pulmonary arterial wedge pressure; PVR, pulmonary vascular resistance; Q, quartile; RAP, right arterial pressure; SSc, systemic sclerosis; TLC, total lung capacity; WHO-FC, WHO Functional Class.