What Is Pulmonary Fibrosis?

Schematic illustration of human lungs

Schematic illustration of human lungs with the different elements on a colored background.

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. Pulmonary fibrosis can develop slowly or quickly. There is no cure. Many people with the disease live only about three to five years after diagnosis.

How is Pulmonary Fibrosis Detected

chest_xrayYour doctor will do a physical exam. Your doctor may ask about your history of smoking, things in the air at your job that could irritate your lungs, your history of drug use, other medical conditions, your family’s medical history, and your hobbies. Other tests for pulmonary fibrosis may include:

  1. Chest X-ray
  2. Lung function tests to find out how much lung damage you have
  3. Blood tests to check for oxygen levels in your bloodstream and for possible infections
  4. Bronchoalveolar lavage, a test that removes and examines cells from the lung.  The test is done during bronchoscopy, a special examination of the lung.
  5. Lung biopsy, done during bronchoscopy or as a surgical procedure. The biopsy removes a sample of lung tissue for your doctor to study.
  6. CT scan
  7. Exercise testing to find out how well your lungs move oxygen and carbon dioxide in and out of your bloodstream.

Treatment of Pulmonary Fibrosis

PrednisoneThere is no cure for pulmonary fibrosis. Current treatments are aimed at preventing more lung scarring, relieving symptoms and helping you stay active and healthy. Treatment cannot fix lung scarring that has already occurred.

Your doctor may prescribe prednisone to reduce inflammation. Prednisone is a corticosteroid. It can have serious side effects. Other medicines sometimes given for pulmonary fibrosis are azathioprine and cyclophosphamide, which suppress the immune system. They also can cause serious side effects.

These treatments may help prevent further scarring and increase survival time in some patients, but they don’t work for everyone with pulmonary fibrosis.

  1. Oxygen therapy may be prescribed if the amount of oxygen in your blood gets low. Supplemental oxygen therapy may help reduce your shortness of breath and make it easier for you to stay active.
  2. Pulmonary rehabilitation is a program that teaches you about your lung disease, how to exercise and how to manage your disease, and provides support and counseling.
  3. Lung transplant may be recommended for you if you are younger than 65, have no other medical problems and are not being helped by medicines.