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What is Pulmonary Fibrosis?

The word "pulmonary" means “lung” and the word "fibrosis" means scar tissue – similar to scars that you may have on your skin from an old injury or surgery. So, in its simplest sense, pulmonary fibrosis (PF) means scarring in the lungs. But, pulmonary fibrosis is more serious than just having a scar in your lung. In PF, the scar tissue builds up in the walls of the air sacs of the lungs, and eventually the scar tissue makes it hard for oxygen to get into your blood. Low oxygen levels (and the stiff scar tissue itself) can cause you to feel short of breath, particularly when walking and exercising.

Also, 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 falls into an even larger group of diseases called the “interstitial lung diseases.” Some interstitial lung diseases don't include scar tissue. When an interstitial lung disease includes scar tissue in the lung, we call it pulmonary fibrosis.

It can be challenging to figure out what causes pulmonary fibrosis. Sometimes doctors are able to identify one or more causes of the disease, but it is also common to end up without an answer, despite completing a large number of medical tests. Diseases where a specific cause is not identified are often called "idiopathic" - meaning the cause is not identified or the disease occurs spontaneously.

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