Interstitial lung disease (ILD) is a group of lung pathologies characterized by marked scarring or fibrosis of the bronchioles and alveolar sacs within the lungs1,2. The increase in fibrotic tissue in ILD prevents oxygenation and free exchange of gases between the pulmonary capillaries and the alveolar sacs1,2. Among these diseases, the most common are idiopathic pulmonary fibrosis (IPF), other interstitial pneumonias, and connective tissue and autoimmune diseases such as scleroderma, rheumatoid arthritis and lupus.
Pulmonary hypertension associated with interstitial lung disease (PH-ILD) is a type of pulmonary hypertension (PH) that arises in patients with ILD, which can shorten their life expectancy. It is estimated to affect at least 15% of patients with early-stage PID and may affect up to 86% of patients with more severe PID3. Both PH and PID damage the lungs, and PH also affects the heart.
