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 death1

Early identification and intervention are key to changing the course of the disease2

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

  • It is most commonly seen in SSc, accounting for 75% of CTD-PAH cases4
  • Approximately 1 in 10 patients with SSc are estimated to have PAH5

PAH is a leading cause of death in patients with SSc, accounting for >50% of deaths in SSc-PAH patients6

Early identification of PAH in patients with SSc is critical

Early recognition of PAH in SSc is difficult; symptoms at disease onset are mild and the complex nature of SSc makes interpretation of fatigue and breathlessness challenging8

However, early evaluation can lead to prompt referral and confirmation – and improved patient outcomes

When evaluating a patient with SSc, it is vital to look at the complete picture, including:

1. Patient history

  • Increasing breathlessness, reduced exercise tolerance, chest pain, ankle swelling and presyncopal symptoms could indicate the presence of PAH in your SSc patients9
  • These symptoms should prompt immediate consideration of PAH in your SSc patient10

2. Clinical examination

  • Look for features of right ventricular (RV) dysfunction10
  • Examine for an RV heave and raised jugular venous pressure (JVP)10,11

3. Non-invasive screening tests

  • Right heart echocardiography12
  • DLCO12
  • Circulating N-terminal pro-B-type natriuretic peptide (NT-proBNP)12

Screening model helps the path to referral

Screening SSc patients for PAH can lead to early diagnosis and improvements in long-term outcomes compared with diagnosis during routine care13

It is therefore recommended that all asymptomatic patients with SSc are screened for PAH annually and referred for right heart catheterisation (RHC) if PAH is suspected12


Referral for RHC can be recommended, depending on the echocardiographic probability of PAH

Adapted from Galié et al. 201612 & Galié 200915

DLCO: in-depth clinical follow-up is necessary for patients with a moderate to severe decrease.12

NT-proBNP (ng/l):12


PAH remains a major cause of mortality in SSc10

Unlike idiopathic PAH, clinicians have the opportunity to actively screen for SSc-PAH and refer to a specialist PH centre to confirm diagnosis with RHC14

Screening for PAH in patients with SSc can lead to an increase of 47% in survival after 8 years vs those detected during routine clinical practice14


Adapted from Humbert et al. 201114



Want to know more? Contact our medical team!

Mathijs Kruk

Medical Scientific Liaison Pulmonary Hypertension NL
+31 6 1238 17 11
[email protected]

Rob Janssen, PhD

Medical Advisor Pulmonary Hypertension NL
+31 6 53 72 73 03
[email protected]


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


  1. Galiè N, et al. Eur Heart J 2010; 31(17):2080–2086
  2. National Pulmonary Hypertension Centres of the UK and Ireland Heart 2008; 94:i1–i41
  3. Rhee RL, et al. Am J Respir Crit Care 2015; 192(9):1111–1117
  4. Zanatta E, et al. Exp Biol Med (Maywood) 2019; 244(2):120–131
  5. Kiely DG, et al. Eur Heart J 2019; 21(Suppl K):K9–20
  6. Kolstad KD, et al. Chest 2018; 154(4):862–871
  7. Badesch DB, et al. Chest 2010; 137(2):376–387
  8. Morrisroe K, et al. Arthritis Res Ther 2017; 19(1):42
  9. McGoon M, et al. Chest 2004; 126(Suppl 1):14S–34S
  10. Chaisson N, Hassoun P. Chest 2013; 144(4):1346–1356
  11. Ryan J, et al. Can J Cardiol 2015; 31(4):391–406
  12. Galiè N, et al. Eur Heart J 2016; 37(1):67–119
  13. Coghlan JG, et al. Ann Rheum Dis 2014; 73(7):1340–1349
  14. Humbert M, et al. Arthritis Rheum 2011; 63(11):3522–353
  15. Galiè N, et al. Eur Heart J 2009; 30(20):2493-2537.)

CP-207909 - 29-jan-2021