Interstitial lung disease (ILD) is a group of lung disorders characterized by pronounced scarring or fibrosis of the bronchioles and alveolar sacs within the lungs1,2. The increase in fibrotic tissue in ILD prevents adequate oxygenation and gas exchange 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 occurs in patients with ILD and may shorten life expectancy. It is estimated to affect at least 15% of patients with early-stage ILD and up to 86% of those with more severe ILD3. Both PH and ILD damage the lungs, and PH also affects the heart.
