Pulmonary arterial hypertension (PAH)

PAH is a potential complication of scleroderma, that can affect your lungs.1 Here you can find out what it is and what to be aware of. 

2 min read

Woman with scleroderma outside next to tree

PAH happens because fibrosis (scarring) narrows the small blood vessels in the lungs. This causes the blood pressure to rise in the pulmonary artery (the big blood vessel that carries blood to the lungs), as it is trying to force blood through narrower blood vessels.1,2

The result of PAH is that less blood gets to the lungs, so less oxygen can get from them into the bloodstream. The heart tries to fix the problem by making its right side, which pumps blood to the lungs, work much harder.1,2

Symptoms of PAH may include:2

  • Getting out of breath doing simple tasks like climbing stairs.
  • Feeling very tired when going about your normal day.
  • Feeling dizzy.
  • Chest pain.
  • Swollen legs.
  • Bluish colour to your lips and skin (cyanosis).
  • Racing pulse or heart palpitations.

Your doctor will be monitoring you regularly to check on the health of your heart and lungs. If you do develop PAH, your doctor will help you manage it and recommend a treatment that suits you. Some treatments for the condition are already available.3

Contact your doctor

Feeling short of breath doesn't mean you have PAH, but you must tell a doctor.4

The facts:

1 in 4
people with severe scleroderma might develop serious lung symptoms that affect how they go about their life.3
Up to 50%
of people who will be affected by lung problems develop symptoms within 3 years of having scleroderma.3,5-7
of people with scleroderma are affected by PAH.8