Diagnosing and Treating Pulmonary Fibrosis | American Lung Association

Diagnosing and Treating Pulmonary Fibrosis

Top 5 Questions for Your Doctor

See 5 important questions to ask your healthcare provider about pulmonary fibrosis.

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How Is Pulmonary Fibrosis Detected?

There are many types of lung disease that can cause pulmonary fibrosis. It is important that your doctor refers you to a specialist to try and make the right diagnosis. Your 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 medication history, other medical conditions, your family's medical history, and your hobbies.

Other tests for pulmonary fibrosis may include:

  • Chest X-ray
  • Breathing tests to find out how much lung damage you have
  • Blood tests to check for oxygen levels in your bloodstream, autoimmune disease, and for possible infections
  • High resolution CT scan of chest (HRCT)
  • Lung biopsy if diagnosis not able to be made by HRCT. The biopsy is done as a surgical procedure and removes small pieces of lung tissue to find a diagnosis.
  • Exercise testing to find out how well your lungs move oxygen and carbon dioxide in and out of your bloodstream.

How Is Pulmonary Fibrosis Treated?

There is no cure for pulmonary fibrosis. Current treatments, including oxygen therapy, are aimed at slowing the course of the disease, relieving symptoms and helping you stay active and healthy. Treatment cannot reverse lung scarring that has already occurred.

Treatments include:

Medicine. Depending on the type of pulmonary fibrosis you have, there may be medications to treat you. Only your medical provider can determine if any medications may be beneficial for you.

Many people diagnosed with pulmonary fibrosis will be prescribed a corticosteroid (prednisone) to reduce inflammation. Corticosteroids can have some serious side effects, especially if used long-term, and need to be carefully monitored. Corticosteroids are not indicated for all types of pulmonary fibrosis. Other medicines sometimes prescribed to reduce inflammation are azathioprine and cyclophosphamide, which suppress the immune system.

The FDA has approved two drugs for one type of pulmonary fibrosis, idiopathic pulmonary fibrosis IPF. They are nintedanib (Ofev®) and pirfenidone (Esbriet®, Pirfenex®, Pirespa®). Both have been shown to slow the progressive decline in lung function in patients with IPF.  These drugs are approved for patients with mild, moderate, and severe IPF. 

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.

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.

Lung transplant evaluation should be recommended early after diagnosis by your physician. To be considered eligible for a lung transplant, you will need to be in good physical condition besides your lung function, have no other life-threatening illnesses, and be willing to accept the risks of surgery and be compliant with subsequent medical treatment.

You may be a candidate for a clinical trial of a new pulmonary fibrosis treatment. Information on clinical trials can be found at ClinicalTrials.gov.

Learn about living with pulmonary fibrosis.

    Approved by Scientific and Medical Editorial Review Panel. Last reviewed October 31, 2016.

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