What causes PAH?

A number of things can cause PAH. These include inherited genetic conditions, other diseases, and outside factors. There are also cases where the cause of PAH is not known.

PAH Cause Description
Idiopathic PAH (IPAH)1 Idiopathic means the cause is not known
Familial or heritable PAH (HPAH)1 This means the disease runs in a family
Connective tissue disorders (CTD)1 PAH associated with CTD
Congenital heart disease (CHD)1 PAH associated with CHD with repaired shunts
Other causes1 PAH associated with other diseases and conditions

The connection between scleroderma and PAH2,3

PAH can be associated with connective tissue disease (referred to as PAH-CTD). Scleroderma is a type of connective tissue disease. People with scleroderma have a higher risk for developing PAH. About 8% to 12% of patients with scleroderma develop PAH. PAH may be the result of the same processes that cause scleroderma.

  • In scleroderma, the blood vessels are injured or damaged. This causes a build-up of connective tissue and causes the blood vessels to narrow (become less wide). These changes make it difficult for blood to flow. As a result, your heart must work harder to pump blood from the right side of your heart through the arteries of your lungs, which lowers the supply of oxygen to your body

The 3 pathways that PAH treatments target4

Blood vessels in your lungs may be affected when there is either too much or too little of certain natural chemicals:

  • Endothelin
  • Nitric oxide
  • Prostacyclin

There are PAH treatments available that target each of these pathways:

About Uptravi PAH Treatment Target Pathways

How UPTRAVI works

UPTRAVI targets the prostacyclin pathway. Your body may not make enough of the chemical prostacyclin. This can cause the blood vessels in your lungs to narrow.