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Learn About Pulmonary Fibrosis

Pulmonary fibrosis is one of a family of related diseases called interstitial lung diseases that can result in lung scarring.

Key Facts

  • There is no cure for pulmonary fibrosis, but new treatments can slow the worsening of the disease in some people
  • Scarred lung tissue makes it is harder to breathe
  • Once the lung tissue becomes scarred, the damage cannot be reversed

What Is Pulmonary Fibrosis?

Pulmonary fibrosis is a disease marked by scarring in the lungs. Tissue deep in the lungs becomes thick, stiff and scarred. The scarring is called fibrosis. As the lung tissue becomes scarred, it interferes with a person's ability to breathe.

In some cases, the cause of pulmonary fibrosis can be found. But most cases of pulmonary fibrosis have no known cause. These cases are called idiopathic pulmonary fibrosis.

How Pulmonary Fibrosis Affects Your Body

In pulmonary fibrosis, the tissue inside and between the air sacs in the lungs becomes scarred. When the scar forms, the tissue becomes stiff and thicker. This makes it harder for oxygen to pass through the walls of the air sac into the bloodstream. Once the lung tissue becomes scarred, the damage cannot be reversed.

How Serious Is Pulmonary Fibrosis?

About 140,000 Americans have been diagnosed with pulmonary fibrosis. It is most likely to affect people ages 50 to 75.

Pulmonary fibrosis can develop slowly or quickly. In some people, the disease stays the same for years. Usually, a person's breathing symptoms become worse over time. A person with pulmonary fibrosis eventually may be short of breath even at rest.

There is no cure. Many people with the disease live only about three to five years after diagnosis.

Pulmonary fibrosis can lead to other medical problems, including collapsed lung, lung infections, blood clots in the lungs and lung cancer. As the disease gets worse, it can lead to respiratory failure, pulmonary hypertension and heart failure. A person with pulmonary fibrosis may need supplemental oxygen to help with breathing.

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