Understanding scleroderma-related PAH

Understanding scleroderma-related PAH

 
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Scleroderma is a type of connective tissue disease (CTD). Some patients with scleroderma may also develop PAH.1-3
People with scleroderma have a higher risk for developing PAH. About 5% to 12% of patients with scleroderma develop PAH.1-4
When PAH arises as a complication of CTD such as scleroderma, it may progress rapidly, which is why timely diagnosis and treatment are important.5
The connection
between PAH and
scleroderma
The connection between
PAH and scleroderma
PAH may be the result of the same processes that cause scleroderma. In scleroderma, the blood vessels are injured or damaged. This causes a build-up of connective tissue and causes the blood vessels to narrow. These changes make it difficult for blood to flow. As a result, your heart must work harder to pump blood from the right side of your heart through the arteries of your lungs, which lowers the supply of oxygen to your body.1-3
Find out if there is an additional treatment angle you could try to help delay PAH progression6
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References: 1. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25)(suppl D):D34-D41. 2. Pulmonary Hypertension Association. Scleroderma and Pulmonary Hypertension. https://phassociation.org/patients/aboutph/diseases-and-conditions-associated-with-ph/scleroderma. Accessed November 27, 2017. 3. Scleroderma Foundation. Pulmonary Hypertension in Scleroderma. Danvers, MA: Scleroderma Foudation; 2017. 4. Galie N, Eur Heart J. European Heart Journal. 2016;37(67-119). 5. McLaughlin V, Humbert M, Coghlan G, Nash P, Steen V. Pulmonary arterial hypertension: the most devastating vascular complication of systemic sclerosis. Rheumatology (Oxford). 2009;48(Suppl 3):iii25-31. 6. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. Circulation. 2009;119(16):2250-2294.
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