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 tiny air sacs (alveoli) in the lungs. The scarring is called fibrosis. It causes the tissues 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. 

Closeup of bronchiole and alveoli showing normal gas exchange.

Closeup of bronchiole and alveoli showing pulmonary fibrosis.

What causes pulmonary fibrosis?

Most of the time, healthcare providers don’t know the cause of pulmonary fibrosis. Things that can increase your risk of getting pulmonary fibrosis are:

  • Smoking

  • Certain viral infections 

  • Pollution, such as silica and metal dusts, bacteria, and 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 healthcare provider will ask about your health history and current symptoms. He or she will do a physical exam. You may need diagnostic tests, such as a chest X-ray, a CT scan of the lungs, and blood tests. Other tests may include:

  • Lung function tests. These tests find out how well your lungs work. A common test is spirometry.

  • Bronchoalveolar lavage. A bronchoalveolar lavage looks at cells from your lungs. It is done during a bronchoscopy. A brochoscope is a special tool that lets your healthcare provide see inside your lungs. He or she can also use it to take small samples of tissue for testing.

  • Lung biopsy. A small sample of lung tissue is taken and then looked at under a microscope. A biopsy is done during 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 healthcare provider will discuss possible treatments with you. These can include:

  • Medicines. These can help reduce inflammation in the lungs. They also can suppress your body’s immune system and help lessen scarring.

  • Supplemental oxygen. This can help more oxygen get into your 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.