What is Pulmonary Fibrosis?

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What Does "Pulmonary Fibrosis" Mean?

The word “pulmonary” means lung and the word “fibrosis” means scar tissue— similar to scars that form on the skin from an old injury or surgery. So, in its simplest sense, pulmonary fibrosis (PF) means scarring in the lungs. Over time, the scar tissue blocks the movement of oxygen from inside the tiny air sacs in the lungs into the bloodstream. Low oxygen levels (and the stiff scar tissue itself) can cause people who have pulmonary fibrosis to feel short of breath, particularly when walking and exercising.

Interstitial Lung Disease and Pulmonary Fibrosis

Pulmonary fibrosis isn’t just one disease. It is a family of more than 200 different lung diseases that all look very much alike. The PF family of lung diseases is part of an even larger group of diseases called interstitial lung diseases (also known as ILD), which includes all of the diseases that have inflammation and/or scarring in the lung. In ILDs, it is the walls of the air sacs of the lung, and the tissue and space around these air sacs, where the injury and damage occur. Some interstitial lung diseases don’t include scar tissue. When an interstitial lung disease does include scar tissue in the lung, we call it pulmonary fibrosis.

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Pulmonary Fibrosis and Interstitial Lung Disease Statistics

No one is certain how many people are affected by PF. One recent study estimated that idiopathic pulmonary fibrosis (or IPF, which is just one of more than 200 types of PF) affects 1 out of 200 adults over the age of 70 in the United States. There are over 200,000 Americans living with PF today. Approximately 50,000 new cases are diagnosed each year and as many as 40,000 Americans die from IPF each year.

Educational Materials

Find reliable information and trusted resources that can help you learn about pulmonary fibrosis and live better with PF.

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  • Pulmonary Fibrosis Quick Facts

    This one-page document provides quick facts about pulmonary fibrosis, including symptoms, causes, treatment options, and more.
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  • Pulmonary Fibrosis Information Guide

    Our comprehensive guide provides reliable information about pulmonary fibrosis, the diagnostic process, treatment options, and more.
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PFF Help Center

For those living with pulmonary fibrosis, obtaining the most accurate and current information can be a frustrating and challenging task. Let us help you find your answers.