SSC and PAH
SSC and PAH
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
Early identification and intervention are key to changing the course of the disease
PAH is a severe and often fatal complication of CTD:
- It is most commonly seen in SSc, accounting for 75% of CTD-PAH cases
- Approximately 1 in 10 patients with SSc are estimated to have PAH
PAH is a leading cause of death in patients with SSc, accounting for >50% of deaths in SSc-PAH patients
Adapted from Badesch et al. 2010
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Contact
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]
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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
CP-192318 - November 2020
