Table 1 Demographics and patient characteristics at baseline
ParameterValue
Male/female, n (%)9 (17%)/44 (83%)
Age, years (mean (SD))63 (13) (range 22–79)
Weight, kg (mean (SD))67 (13) (range 40–99)
Caucasian/Asian51 (96%)/2 (4%)
Aetiology, n (%):
    Limited systemic sclerosis29 (55%)
    Diffuse systemic sclerosis13 (25%)
    Mixed connective tissue disease6 (11%)
    Systemic lupus erythaematosus5 (9%)
Time from CTD diagnosis, weeks (mean (SD))431 (503) (range 0–2227)
Time from PAH diagnosis, weeks (mean (SD))45 (66) (range 1–236)
Signs of right heart failure, n (%)8 (15%)
Patients with at least one digital ulcer, n (%) (n = 50)15 (30%)
Right heart catheterisation:
    Mean pulmonary arterial pressure, mmHg (mean (SD))39.5 (12.6)
    Cardiac index, litres/min/m2 (mean (SD); n = 49)2.9 (0.9)
    Mean pulmonary capillary wedge pressure, mmHg (mean (SD); n = 51)10.1 (4.4)
    Pulmonary vascular resistance, dyn/s/cm–5 (mean (SD); n = 47)559.4 (371.5)
Total lung capacity, % (mean (SD); n = 46)80.8 (18.3)
Forced vital capacity, % of predicted (mean (SD))85.9 (23.6)
Concomitant treatment, n (%):
    Antithrombotic agents*41 (77%)
    Antacids/drug for treatment of peptic ulcer and flatulence40 (75%)
    Calcium channel blockers32 (60%)
    Corticosteroids†30 (57%)
    Diuretics28 (53%)
  • Unless otherwise stated, n = 53.

  • *Mostly acenocoumarol, warfarin, or acetylsalicylic acid. †Mostly prednisolone.

  • CTD, connective tissue disease; PAH, pulmonary arterial hypertension.