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]
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]
Contact
Want to know more? Contact our medical team!
MATHIJS KRUK
Medical Scientific Liaison Pulmonary
Hypertension NL
+31 6 1238 17 11
mkruk1@its.jnj.com
ROB JANSSEN, PHD
Medical Advisor Pulmonary
Hypertension NL
+31 6 53 72 73 03
rjansse9@its.jnj.com
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Abbreviations
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
