What Is IPF?


Idiopathic pulmonary fibrosis (IPF) is a serious disease that causes irreversible scarring of the lungs and progresses, or gets worse, over time. This scarring makes it difficult to breathe and—eventually—the lungs can’t inhale enough oxygen for the body to function properly.

What Does “IPF” Stand for?

“Idiopathic” means that the cause of the disease is unknown.

“Pulmonary” means it impacts the lungs.

“Fibrosis” means scarring.

How Is IPF Related to ILD?

IPF is one of the most common forms of interstitial lung disease (ILD). Many types of ILD have a known cause, but sometimes the cause is unknown. When the cause of ILD is unknown, it’s called idiopathic interstitial pneumonia. IPF is one type of idiopathic interstitial pneumonia.

Unlike other types of ILD with scarring that may or may not get worse over time, IPF causes progressive lung scarring, which means scarring of the lungs that gets worse over time. Sometimes this is called worsening fibrosis. Because IPF may progress at varying rates, it’s important to take action early.

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Read about your options for managing IPF, including a treatment that may slow the progression of the disease.
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