What Is Pulmonary Arterial Hypertension

Scleroderma Can Also Affect Your Lungs

Systemic sclerosis, or scleroderma, is not just a skin condition. It can also cause serious lung complications such as pulmonary arterial hypertension, or PAH for short.^1,2

PAH is a disease of high blood pressure in the lungs. It can occur in people with different connective tissue diseases but PAH is most common in people with scleroderma.^1,3

Arteries that are healthy allow blood to flow easily through the vessels.⁴

In , the blood pressure in the arteries of your lungs is high.⁴

Artery with
effects
from

The that carry blood
from your heart to your lungs have
become stiff and narrow.⁴

Artery with
worsening
signs of

The that carry blood
from your heart to your lungs have
become more stiff and narrow.⁴

Therefore, it is difficult for blood to flow through your lungs.⁴

This can force your heart to have to work harder than normal.⁴

Scleroderma Can Increase Your Risk for PAH

Although scleroderma and PAH are 2 separate conditions, inflammation may play an important role in both. Scleroderma can cause inflammation, which may then increase your chances of developing PAH.^3,5

Signs and Symptoms of PAH

Noticeable signs of PAH may start out mild but can get worse over time. They include^2,3,6,7:

Fainting

Light-headedness

Constant tiredness

Shortness of breath

Chest pain

Swelling of the abdomen
(also called edema)

Swelling of the ankles or legs
(also called edema)

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.^7,8

If you are diagnosed with PAH, medicines are available that may
be able to help. Talk to your doctor about screening for PAH.⁴

Track how you are feeling

Are changes in how you feel being caused by scleroderma, PAH associated with scleroderma, or something else?

It can be difficult to tell, but tracking how you feel every day can help you discuss your health with your healthcare team.

Take Charge of Your Health

If you have scleroderma, experts recommend annual screening for PAH.⁸

Have Questions About PAH?

See some of the most commonly asked questions about scleroderma and PAH.

Find answers

References: 1. 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. 2. 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. 3. Chaisson NF, Hassoun PM. Systemic sclerosis-associated pulmonary arterial hypertension. CHEST. 2013;144(4):1346-1356. 4. 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 5. 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. 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. Hayes GB. Pulmonary Hypertension: A Patient’s Survival Guide. 5th ed. Silver Spring, MD: Pulmonary Hypertension Association; 2012. 8. 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.

What Is Pulmonary Arterial Hypertension?