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Frequently Asked Questions

Here are answers to some of the most frequently asked questions about scleroderma and pulmonary arterial hypertension (PAH).

There are 2 main types of systemic sclerosis, or scleroderma. Localized scleroderma primarily affects the skin, while systemic sclerosis affects the skin, blood vessels, and major organs.1

Scleroderma is not just a skin condition. It can also cause serious lung complications, such as pulmonary arterial hypertension (PAH).2,3 PAH is a disease of high blood pressure in the lungs.4

Arteries that are healthy allow blood to flow easily through the vessels. In PAH, the blood pressure in the arteries of your lungs is high. This is because the arteries that carry blood from your heart to your lungs have become stiff and narrow, making it difficult for blood to flow through your lungs. This can force your heart to have to work harder than normal.5

Yes, PAH can be life-threatening, especially for people with scleroderma.6 PAH was the leading cause of death—ahead of cancer, infections, and other causes—in a registry7* of people with PAH associated with systemic sclerosis.8

While there is no cure for PAH, treatments are available.6,9 So, if you find out you also have PAH, discuss your options with your doctor.

*A registry is a collection of standardized information about a group of patients who share a particular disease or condition.

Although scleroderma and PAH are 2 separate conditions, inflammation may play an important role in both.10 Scleroderma can cause inflammation, which may then increase your chances of developing PAH.5,10,11

PAH can occur in people with different connective tissue diseases but is most common in people with scleroderma.6 Five percent to 12% of people with scleroderma will also develop PAH.12

Noticeable signs of PAH may start out mild but can get worse over time. They include light-headedness; fainting; constant tiredness; shortness of breath; chest pain or discomfort; and swelling of the ankles, legs, or abdomen (also called edema).4,5,7,13 But PAH can begin before you notice it, so screening every year is recommended by PAH experts and may help to detect it early.12,14-16

Recommendations for annual PAH screening of people with scleroderma are included in the 2015 European Society of Cardiology/European Respiratory Society Guidelines, proceedings from the 6th World Symposium of Pulmonary Hypertension, and results of a consensus survey of experts in the field of PAH and sclerosis.11,13,14

Some signs of PAH are silent, so you may not see or feel them. Blood work, pulmonary function tests, or an ultrasound of your heart can reveal signs of PAH that indicate the need for diagnostic testing. These screening tests can show if you might have PAH.13,14

In order to confirm a PAH diagnosis, a test called right heart catheterization is required. Your doctor may order a right heart catheterization if other tests suggest you might have PAH.13,14

No, there is no cure for PAH. However, regular screening may help to detect PAH early. PAH experts† recommend that you get screened every year. If you are diagnosed with PAH, medicines are available that may be able to help.6,12,14,15

†Recommendations for annual PAH screening of people with systemic sclerosis are included in the 2015 European Society of Cardiology/European Respiratory Society Guidelines, proceedings from the 6th World Symposium of Pulmonary Hypertension, and results of a consensus survey of experts in the field of PAH and scleroderma.11,13,14

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References: 1. Johns Hopkins Medicine. Types of scleroderma. Accessed November 11, 2021. https://www.hopkinsmedicine.org/health/conditions-and-diseases/scleroderma/types-of-scleroderma 2. Fischer A, Bull TM, Steen VD. Practical approach to screening for scleroderma-associated pulmonary arterial hypertension. Arthritis Care Res (Hoboken). 2012;64(3):303-310. 3. Mandras SA, Ventura HO, Corris PA. Breaking down the barriers: why the delay in referral for pulmonary arterial hypertension? Ochsner J. 2016;16(3):257-262. 4. Chaisson NF, Hassoun PM. Systemic sclerosis-associated pulmonary arterial hypertension. CHEST. 2013;144(4):1346-1356. 5. Pulmonary Hypertension Association. Pulmonary hypertension and scleroderma brochure. Published 2018. Accessed November 11, 2021. https://phassociation.org/wp-content/uploads/2021/08/PHA-Scleroderma.pdf 6. Vachiéry J-L, Gaine S. Challenges in the diagnosis and treatment of pulmonary arterial hypertension. Eur Respir Rev. 2012;21(126):313-320. 7. Workman TA. Engaging patients in information sharing and data collection: the role of patient-powered registries and research networks. US Department of Health and Human Services, Agency for Healthcare Research and Quality. 2013. AHRQ Report No.: 13-EHC124-EF. 8. Kolstad KD, Li S, Steen V, Chung L; PHAROS Investigators. Long-term outcomes in systemic sclerosis-associated pulmonary arterial hypertension from the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry (PHAROS). CHEST. 2018;154(4):862-871. 9. Benza RL, Kanwar MK, Raina A et al. Development and validation of an abridged version of the REVEAL 2.0 risk score calculator, REVEAL Lite 2 , for use in patients with pulmonary arterial hypertension. CHEST. 2021;159(1):337-346. 10. Mathai SC, Hassoun PM. Pulmonary arterial hypertension in connective tissue diseases. Heart Fail Clin. 2012;8(3):413-425. 11. Zanatta E, Polito P, Famoso G, et al. Pulmonary arterial hypertension in connective tissue disorders: pathophysiology and treatment. Exp Biol Med. 2019;244(2):120-131. 12. Galiè N, Humbert M, Vachiery J-L, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Eur Heart J. 2016;37(1):67-119. 13. Hayes GB. Pulmonary Hypertension: A Patient’s Survival Guide. 5th ed. Silver Spring, MD: Pulmonary Hypertension Association; 2012. 14. Khanna D, Gladue H, Channick R, et al. Recommendations for screening and detection of connective-tissue disease associated pulmonary arterial hypertension. Arthritis Rheum. 2013;65(12):3194-3201. 15. Frost A, Badesch D, Gibbs JSR, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019;53(1):1801904. doi:10.1183/13993003.01904-2018 16. Hachulla E, Launay D, Mouthon L, et al; French PAH-SSc Network. Is pulmonary arterial hypertension really a late complication of systemic sclerosis? CHEST. 2009;136(5):1211-1219.