7 Facts About Scleroderma-Related PAH

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Scleroderma (SSc) is a rare but progressive autoimmune disease that mainly affects women between the ages of 20 and 50, although men and children can also develop the disease. Many patients who have scleroderma also develop pulmonary arterial hypertension (PAH).

1. According to the Scleroderma Foundation, there are an estimated 300,000 scleroderma patients in the U.S.

Three possible risks for scleroderma. Read about them here.

2. It is estimated that between 8 and 30 percent of scleroderma patients will develop PAH.

Read about seven common complications of scleroderma here. 

3. There are about 200,000 new cases of PAH reported each year in the U.S.

Read more about overlapping medical conditions in scleroderma here.

4. Pulmonary arterial hypertension (PAH) is categorized into four groups, with one being the mildest and four being the most severe.

Scleroderma patients with PAH may benefit from PDE-5 inhibitors as early therapy. Read more here.

5. The relationship between PH and scleroderma is because of the nature of scleroderma—it attacks the connective tissues in the body’s major organs.

Scleroderma more severe in men and leads to PAH quicker, study finds. Read more here.

6. While the skin is predominantly affected by scleroderma, many patients also struggle with lung problems.

Read more about how scleroderma and PH often co-exist.

7. The coexistence of PH and scleroderma is due to the thickening of connective tissue around blood vessels. When blood vessels are narrowed in the lungs, the right ventricle of the heart must work harder to pump blood to the lungs.

Read about seven common complications of scleroderma here. 


Scleroderma News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or another qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.