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TYVASO and TYVASO DPI are approved for the treatment of pulmonary hypertension associated with interstitial lung disease (PH-ILD; WHO Group 3) to improve the ability to exercise.


PH-ILD stands for pulmonary hypertension associated with interstitial lung disease, a rare, serious, and progressive disease. Breaking down the parts of the name “PH-ILD” can give you a good picture of how they fit together.


PH (Pulmonary Hypertension)

Pulmonary means it affects the lungs.
ension means high blood pressure.

…So, pulmonary hypertension is high blood pressure in the lungs.


ILD (Interstitial Lung Disease)

Interstitial Lung Disease is a group of serious, progressive lung disorders that can damage the lungs and make it harder to breathe.

Some examples of ILD include:

  • Idiopathic pulmonary fibrosis (IPF) or pulmonary fibrosis (PF)
  • Combined pulmonary fibrosis and emphysema (CPFE)
  • Connective tissue disease (CTD)

Putting it all together

Sometimes, people with ILD can also have PH, which is why you see the terms put together as one. PH is often hard to diagnose because its symptoms, like shortness of breath, fatigue, and coughing, can seem very similar to ILD. It is not always clear that there is something else going on.

In fact, in IPF (which is one type of ILD), PH is present in up to 15% of patients at the early stages of IPF—or even at diagnosis.

It’s important to be checked for PH regularly because PH can appear at any time as your ILD progresses.

Learn about how ILD progresses to PH-ILD.