What is pulmonary fibrosis and how is it treated?
Ask the doctor
Q. I was recently diagnosed with pulmonary fibrosis. What kind of treatment is available? Can anything be done to keep it from worsening? What is the life expectancy?
A. Pulmonary fibrosis is a condition in which there is significant or widespread scarring in the lungs.
The disease has many causes or triggers. These include medications, immune diseases, and exposure to inhaled toxins such as asbestosis. Sometimes no trigger is ever found.
Scar tissue in the lungs is usually permanent, just as it is in other parts of the body. There is currently no reliable way to treat, cure, or halt the progression of pulmonary fibrosis.
Therapy can be used to reduce symptoms of breathlessness or coughing. This might include oxygen or anti-anxiety medicines. Other important measures include avoiding pulmonary toxins (such as air pollution), getting routine vaccinations to prevent lung infection, and not smoking. If there is an underlying disease (such as lupus) contributing to lung disease, effective treatment of that disease may slow progression of lung scarring.
Treatments for pulmonary fibrosis may include:
- pulmonary rehabilitation (exercise, chest physical therapy, and education)
- anti-scarring medications including nerandomilast, nintedanib, or pirfenidone
- lung transplantation.
The choice of treatment depends on a number of factors. For example, people with significant liver disease should not receive nintedanib, as this medicine can cause liver injury or worsen existing liver problems.
Researchers are looking for other therapies that can slow or reverse lung scarring. It's worth asking your doctor about clinical trials of promising experimental treatments. You may be able to enroll in a study in your area.
Life expectancy with pulmonary fibrosis varies. Minor amounts of scarring in the lung may have little or no impact on lifespan. People with more severe or progressive disease may have a five-year survival rate as low as 20%. However, a number of factors go into these estimates, and doctors are not very accurate in predicting life expectancy with this condition.
Image: © Liubomyr Vorona/Getty Images
About the Author
Robert H. Shmerling, MD, Senior Faculty Editor, Harvard Health Publishing; Editorial Advisory Board Member, Harvard Health Publishing
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