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]

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 challenging[8]

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 patients[9]
  • These symptoms should prompt immediate consideration of PAH in your SSc patient[10]

2. Clinical examination

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

3. Non-invasive screening tests

  • Right heart echocardiography[12]
  • DLCO[12]
  • 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 care[13]

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 suspected[12]

2015 ESC/ERS guidelines - screening strategy

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

SSC and PAH - Screening - Flowchart
SSC and PAH - Screening - Risk level
Adapted from Galié et al. 2016[^12] & Galié 2009[^15]

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

NT-proBNP (ng/l):[12]

SSC and PAH - NT-proBNP plasma levels

Early referral of SSc-PAH patients is critical

PAH remains a major cause of mortality in SSc[10]

Unlike idiopathic PAH, clinicians have the opportunity to actively screen for SSc-PAH and refer to a specialist PH centre to confirm diagnosis with RHC[14]

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 practice[14]

Impact of screening on survival in patients with SSc-PAH

SSC and PAH - Impact of screening on survival in patients with SSc-PAH

Adapted from Humbert et al. 2011[14]

Early treatment of PAH can delay disease progression and improve long-term outcomes for your patients with SSc-PAH[15][16][17][18]

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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-352777 - January 2023