• Idiopathic pulmonary fibrosis affects mostly people over the age of 50, usually former smokers.
• People may cough, have difficulty breathing, and feel tired.
• Pulmonary rehabilitation, lung transplantation, and drugs such as pirfenidone and nintedanib , are treatments.

(See also Overview of Idiopathic Interstitial Pneumonias and Overview of Interstitial Lung Diseases.)

In idiopathic pulmonary fibrosis, the lungs develop progressive scarring for unknown reasons. There seems to be a genetic component because some families have more than one person affected by the disorder. Specific gene mutations have been identified in some people with idiopathic pulmonary fibrosis.