What Is Idiopathic Pulmonary Fibrosis?
Idiopathic Pulmonary Fibrosis (IPF) is one of the most common forms of interstitial lung disease (ILD).
Interstitial lung diseases primarily affect the air sacs of the lung (alveoli), where the body absorbs oxygen and rids itself of carbon dioxide waste, rather than the tubes (bronchi) that conduct air into the alveoli.
There are no therapies to halt or reverse the course of IPF. There is currently no FDA-approved treatment, and about two-thirds of IPF patients die within five years. Currently, the ultimate treatment for IPF is lung transplantation.
There are many types of ILD. Doctors do not know what causes many of these conditions. Diagnosis often requires obtaining tissue from the lung.
What Happens to People with IPF?
Interstitial lung diseases cause inflammation and scarring in the lungs' air sacs. This leads to two major problems:
- Stiffening of the lungs, which makes it difficult to take a deep breath
- Impairment of the lung’s ability to transport oxygen from the air in the alveoli to the red blood cells, which carry oxygen to the rest of the body
Scarring leads to stiffening of the lungs and inhibits their ability to provide oxygen to other tissues. This makes breathing more difficult and prevents the lungs from supplying enough oxygen to the body. Both men and women can develop IPF—usually between the ages of 40 and 70—but the disease is more common in men.
What Are the Symptoms?
Early symptoms of IPF are similar to those of other lung diseases; dyspnea, shortness of breath, and a dry cough. IPF progresses differently from person to person, but is usually fatal.
What Are the Risk Factors for IPF?
Learn more about the various risk factors for IPF ranging from age, environment, to family history.
What Diagnostic Tests Are Available for IPF?
View a diagnostic tests table for IPF that shows the type study conducted. What is involved? When? Why are they done?