What Is Pulmonary Arterial Hypertension

PAH: Scleroderma and Its Effect on Your Lungs

Systemic sclerosis is not just a skin condition. It can also cause serious lung problems such as pulmonary arterial hypertension, or PAH for short
PAH is a disease of high blood pressure in the lungs. It can happen in people with different connective tissue diseases, but PAH is most common in people with scleroderma
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

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

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

Long view of lungs with healthy arteries

Artery with
effects
from

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

Heart with PAH artery that is becoming stiff and narrow

Artery with
worsening
signs of

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

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

As the muscles work harder to get blood flowing, the walls on the right side of your heart get thicker.

Signs and Symptoms of PAH

Noticeable signs of PAH may start out mild but can get worse over time. They include:

Symptoms and signs of Pulmonary Arterial Hypertension (PAH)

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 hidden, so you may not see or feel them

Blood work, lung function tests, or an ultrasound of your heart can show signs of PAH and the need for diagnostic testing.

People with PAH may have different signs and symptoms. That is why it is important to talk to your rheumatologist about screening for PAH.

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 yearly screening for PAH.*

Have Questions About PAH?

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

Find answers

*Recommendations for yearly PAH screening of people with systemic sclerosis are included in the 2022 European Society of Cardiology/European Respiratory Society Guidelines.

What Is Pulmonary Arterial Hypertension?