Table 1. Prevalence of systemic sclerosis-associated pulmonary arterial hypertension
PrevalenceDiagnostic thresholdRef.
Echocardiogram based
    43%Ppa,sys ≥35 mmHg[34]
    35%Ppa,sys >30 mmHg[42]
    59%Ppa,sys ≥40 mmHg[35]
    16%Ppa,sys >40 mmHg[43]
    26.7%Ppa,sys ≥40 mmHg[37]
    21% limited, 26% diffusePpa,sys >30 mmHg or >35 mmHg[36]
Right heart catheterisation based
    2.93 per million#P̄pa ≥25 mmHg at rest with Ppcw <15 mmHg[44]
    5%P̄pa >25 mmHg at rest with Ppcw ≤15 mmHg[45]
    7%P̄pa >32 mmHg[38]
    16%P̄pa ≥ mmHg at rest with Ppcw ≤12 mmHg[39]
    29%P̄pa ≥20 mmHg or Ppa,sys ≥ 35 mmHg[34]
    12%P̄pa >25 mmHg[40]
    7.85%P̄pa ≥25 mmHg at rest or ≥30 mmHg during exercise, with Ppcw <15 mmHg[41]

  • Ppa,sys: pulmonary artery systolic pressure; P̄pa: mean pulmonary artery pressure; Ppcw: pulmonary capillary wedge pressure. #: general population; : 95% CI 5.7–10.