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. Often, no trigger is found.
As in other parts of the body, scar tissue is usually permanent. There is currently no way to treat, cure or slow the progression of pulmonary fibrosis.
Therapy can be used to reduce symptoms, such as breathlessness or coughing. Avoiding all pulmonary toxins (such as air pollution) and not smoking are essential.
Treating pulmonary fibrosis may include:
- Pulmonary rehabilitation (exercise, chest physical therapy, and education)
- Immune suppression and anti-oxidant therapy (including the drugs azathioprine, glucocorticoids, and acetylcysteine)
- Lung transplantation
The choice of treatment depends on a number of factors. For example, the ideal candidate for immunosuppressive treatment is one who has mild or moderate disease (rather than advanced scarring) and does not have other medical problems that will make this treatment dangerous.
Research is looking to discover therapies that can slow or reverse lung scarring. One recent study found that sildenafil (as found in Viagra and Revatio) improved symptoms in some people with pulmonary fibrosis. Therefore, 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 5-year survival 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.