Understanding Pulmonary Fibrosis

What is Pulmonary Fibrosis?

Pulmonary fibrosis is a disease marked by scarring of the tissue inside and between the air sacs in the lungs. 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.

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

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.

What Causes Pulmonary Fibrosis?

In most cases, there is no known cause for the disease. In these cases, it is called idiopathic pulmonary fibrosis or IPF.

Things that may increase the risk of pulmonary fibrosis include:

  • Cigarette smoking
  • Certain viral infections
  • Exposure to environmental pollutants, including silica and hard metal dusts, bacteria and animal proteins, and gases and fumes
  • The use of certain medicines
  • Genetics. Some families have at least two members who have pulmonary fibrosis.
  • Gastroesophageal reflux disease (GERD). GERD is a condition in which acid from your stomach backs up into your throat. Some people who have GERD may breathe in tiny drops of acid from their stomachs, which may injure the lungs.

Learn more about the symptoms, diagnosis and treatment of pulmonary fibrosis