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Pulmonary Fibrosis

What Is Pulmonary Fibrosis?

Pulmonary fibrosis is an interstitial lung disease. Interstitial lung diseases are a group of conditions that cause inflammation and scarring around the alveoli, or tiny air sacs in the lungs. The scarring is called fibrosis. It causes the tissue in the lungs to get thick and stiff. This makes it hard to take in oxygen. Often the cause is unknown. This is called idiopathic pulmonary fibrosis. 

The exchange of oxygen and carbon dioxide in a normal lung.

The exchange of oxygen and carbon dioxide in a lung with pulmonary fibrosis

What Causes Pulmonary Fibrosis?

Most of the time, the cause of pulmonary fibrosis is unknown. Things that can increase your risk of getting pulmonary fibrosis are:

  • Smoking

  • Certain viral infections

  • Environmental pollutants (for example, silica and metal dusts, bacteria, gases)

  • Certain medicines

  • Genetics. More than one family member may have pulmonary fibrosis.

  • Gastroesophageal reflux disease (GERD)

What Are the Symptoms of Pulmonary Fibrosis?

The symptoms of pulmonary fibrosis include:

  • Difficulty breathing or shortness of breath

  • Cough

  • Chest pain

  • Feeling tired

  • Joint pain

 How Is Pulmonary Fibrosis Diagnosed?

Your health care provider will ask a lot of questions about your medical history and current symptoms. He or she will do a physical examination. Diagnostic tests may include some common tests like a chest X-ray, a CT scan of the lungs, and blood tests. Other tests may include:

  • Lung function tests. These tests are done to see how well your lungs work. A common test is spirometry.

  • Bronchoalveolar lavage. A bronchoalveolar lavage examines cells from the lung. It is done during a bronchoscopy. A bronchoscope if a special tool that allows your health care provider to see inside your lungs. It can also be used to take small samples of tissue for testing.

  • Lung biopsy. A small sample of tissue is taken and then examined under a microscope. A biopsy is done during a bronchoscopy or a surgical procedure.

  • Exercise testing. These tests show how well your lungs work during exercise.

How Is Pulmonary Fibrosis Treated?

Pulmonary fibrosis can’t be cured. Treatment can help control the disease and improve symptoms. Your health care provider will discuss possible treatments with you. These can include:

  • Medications. These can help reduce inflammation in the lungs, suppress the body’s immune system, and help lessen scarring.

  • Supplemental oxygen. This can help increase the amount of oxygen that gets to the blood. Some people will need to use oxygen all the time. Others will only need it when they sleep or exercise.

  • Breathing techniques. These can help you cope with shortness of breath.

  • Pulmonary rehabilitation. This is a program of exercise and education that can help you gain strength and independence.


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