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What is Idiopathic Pulmonary Fibrosis (IPF)?

Idiopathic Pulmonary Fibrosis (IPF) is?

The progressive scarring of the lungs that occurs when air sacs known as alveoli gradually become replaced by fibrotic tissue or scar tissue. As the scar tissue becomes thicker, it leads to stiffness in the lungs, making it difficult to breathe. Pulmonary fibrosis is found in over 200 lung disorders, so it is important for your health care provider to identify the cause of the fibrosis, because different types of fibrosis respond to different treatments.

What’s the prevalence of Pulmonary Fibrosis?

5,000,000 people Worldwide affected
200,000 patients in the United States, of these:
40,000 expire annually


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. 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.

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 250,000 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.

Thank you to Pulmonary Fibrosis Foundation for ALL of their dedication and commitment to the IPF community!

 (ALL above information obtained from Pulmonary Fibrosis Foundation 6.12.22)

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