What is pulmonary hypertension associated with interstitial lung disease?

Interstitial lung disease is a large group of very similar, restrictive lung diseases. Other forms of interstitial lung disease include idiopathic interstitial pneumonia, chronic hypersensitivity pneumonitis, and occupational or environmental lung disease, commonly referred to as pulmonary fibrosis (PF). Pulmonary fibrosis causes thickening and scarring of the air sacs in the lungs. When this happens, pulmonary hypertension can develop and

  • the blood vessels in the lungs can no longer absorb enough oxygen to deliver to the rest of the body
  • the low oxygen level and the stiffness of the lungs can make patients feel short of breath during exercise

Patients who have ILD usually require oxygen therapy, but, unfortunately, few other treatments are available.


idiopathic pulmonary fibrosis

Lung Damage in Pulmonary Fibrosis

As a result of the damage to the lungs caused by pulmonary fibrosis, pulmonary hypertension develops in more than 40% of patients who have the disease.

The pulmonary hypertension requires the right side of the heart to work harder to pump blood through narrowed arteries in the lungs.

To learn more about the iNO-PF Research Study for people with PF, click here.