What is PAH?

A subtype of pulmonary hypertension (PH)

Pulmonary arterial hypertension (PAH) is a rare, progressive disease characterised by elevated pulmonary arterial pressure and vascular resistance that eventually leads to right ventricular failure and death[1]

Early identification and intervention are key to changing the course of the disease[2]

PAH is a severe and often fatal complication of CTD:[3]

  • It is most commonly seen in SSc, accounting for 75% of CTD-PAH cases[4]
  • Approximately 1 in 10 patients with SSc are estimated to have PAH[5]

PAH is a leading cause of death in patients with SSc, accounting for >50% of deaths in SSc-PAH patients[6]

What is PAH? - Different subcategories

Adapted from Badesch et al. 2010[7]

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Medical Scientific Liaison Pulmonary
Hypertension NL
+31 6 1238 17 11


Medical Advisor Pulmonary
Hypertension NL
+31 6 53 72 73 03


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ALT, alanine aminotransferase; AST, aspartate aminotransferase;CHD, congenital heart disease; CI, confidence interval; CTD, connective tissue disease; ERA, endothelin receptor antagonist; ERS, European Respiratory Society; ESC, European Society of Cardiology; HIV, human immunodeficiency virus; HPAH, heritable pulmonary arterial hypertension; HR, hazard ratio; IPAH, idiopathic pulmonary arterial hypertension; LFT, liver function test; PAH, pulmonary arterial hypertension; PH, pulmonary hypertension; PoPH, portopulmonary hypertension; RHC, right heart catheterisation; SSc, systemic sclerosis; TRV, tricuspid regurgitation velocity; ULN, upper limit of normal; 6MWD, six-minute walk distance

CP-192318 - November 2020