Understanding the Risks of Pulmonary Arterial Hypertension

A Potentially Life-threatening Condition

PAH gets worse over time and can be life threatening, especially for people with scleroderma.1

was the leading cause of death, ahead of:
Other causes
in a registry2 of people with PAH associated with scleroderma.3

Who Is at Risk for PAH?

If you have scleroderma, you are at risk for PAH.4

5% to 12% of people with scleroderma will also develop PAH.5

African Americans with scleroderma may develop PAH more frequently than the overall population of people with scleroderma.6

A study from 2003 showed the rate of PAH was higher in 130 African Americans with scleroderma (48%) than in the overall population of 709 people in the scleroderma center (39%).

Other Risk Factors for PAH

Additional factors that can increase your risk include4,7:



Older age at the time of your scleroderma diagnosis



If you have had scleroderma for a long time

Other conditions

Raynaud’s phenomenon
Raynaud’s phenomenon

A condition in which small blood vessels in the hands and toes spasm in the cold

Cutaneous telangiectasias

Spider veins on your skin but may develop anywhere within the body

Digital ischemia
Digital ischemia

Painful pale, white, or blue fingers

Limited cutaneous sclerosis
Limited cutaneous scleroderma

A type of scleroderma in which skin hardening and tightening occurs only on the fingers, hands, forearms, and face

Test results

Presence of certain antibodies

Presence of certain antibodies

Results in the low range on certain pulmonary function tests

Results in the low range on certain pulmonary function tests

What Is PAH?

PAH is a disease of high blood pressure in the lungs that can be more common in people with scleroderma.4,8

Find out more

Talk to Your Doctor

Ask your doctor about annual screening and early diagnosis of PAH.

take action
References: 1. Vachiéry J-L, Gaine S. Challenges in the diagnosis and treatment of pulmonary arterial hypertension. Eur Respir Rev. 2012;21(126):313-320. 2. 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. 3. 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 4. 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. 5. 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. 6. Schachna L, Wigley FM, Chang B, et al. Age and risk of pulmonary arterial hypertension in scleroderma. CHEST. 2003;124(6):2098-2104. 7. Yaqub A, Chung L. Epidemiology and risk factors for pulmonary hypertension in systemic sclerosis. Curr Rheumatol Rep. 2013;15(1):302. 8. Chaisson NF, Hassoun PM. Systemic sclerosis-associated pulmonary arterial hypertension. CHEST. 2013;144(4):1346-1356.