Idiopathic Pulmonary Fibrosis
Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive, debilitating, and usually fatal interstitial lung disease that affects approximately 100,000 people in the U.S. This orphan disease results in fibrotic scarring of the lungs. Current treatment options are limited and poorly tolerated.
Idiopathic means “of unknown cause”, though there are certain risk factors that are associated with a higher incidence of IPF, including:
- Age (> 50)
- Male gender
- Acid reflux
- Family history of IPF
While many patients do not have symptoms early in the course of the disease, as IPF progresses, symptoms can include:
- Persistent dry cough
- Shortness of breath, especially with exertion
- Chest pain
- Loss of appetite and non-intentional weight loss
- Swelling in the legs
There are 2 FDA-approved drugs to treat IPF. While both drugs have been shown to decrease the rate of loss of lung function, neither has demonstrated an impact on improved survival and both are poorly tolerated by many patients.