Pulmonary fibrosis is a lung disease that affects about 200,000 Americans according to the American Thoracic Society. In pulmonary fibrosis, the tissues inside the lungs—the airways and alveoli, or air sacs—become scarred and stiff. This stiffness means the airways cannot contract and expand as easily when taking in air, and cellular damage within the alveoli (air sacs) does not allow oxygen to pass as easily to the bloodstream, also impairing carbon dioxide from passed through as easily to be exhaled. This impairs the body’s ability to take in oxygen.
It is suspected that an exaggeration of the healing response in the lungs is responsible for the scarring involved in pulmonary fibrosis; the excessive scar tissue thus may be an overreaction to a normal process, impairing the body’s ability to take in oxygen and making breathing more effortful.
Pulmonary fibrosis most often develops between age 50 and 75. Pulmonary fibrosis is a disease in the family of interstitial lung diseases, which are diseases causing scarring in the lungs. Because the mechanics of the many diseases are similar, pulmonary fibrosis and interstitial lung disease may be used interchangeably by doctors and patients.
Sometimes, the cause of pulmonary fibrosis can be identified—when it cannot, this is known as idiopathic pulmonary fibrosis. A variety of factors may increase risk of pulmonary fibrosis includes inhaled toxins such as cigarette smoking and exposure to air pollutants, taking certain medicines, genetic factors, and gastroesophageal reflux disease (GERD, or acid reflux).
Pulmonary fibrosis can develop quickly or slowly, and its progression can be difficult to predict. In most cases, the disease progresses quickly, sometimes resulting of shortness of breath even at rest. Conversely, some people may have a more slowly progressing form of the disease and may remain stable for years. Pulmonary fibrosis, in addition to the progressive nature of the disease, can also result in lung collapse, infection, blood clots, and lung cancer. Pulmonary hypertension, respiratory failure, and heart failure can also develop as a result.
Pulmonary fibrosis has no known cure, and the life expectancy following diagnosis can vary widely based on the progression of the disease. The life expectancy following diagnosis is commonly 2-3 years, but maybe longer depending on the individual disease state. Often, no predictions on life expectancy can be made. By following proper treatment and with careful medical supervision, patients with pulmonary fibrosis may be able to extend the length of their life and improve their quality of life. Our next blog post will explore treatment options for pulmonary fibrosis, as well as the symptoms of this disease.
The symptoms of pulmonary fibrosis are caused by excessive scarring within the lungs, the hallmark feature of pulmonary fibrosis or interstitial lung disease. The scarring causes alveoli or air sacs, and airways to stiffen, resulting in the lungs being unable to deliver oxygen efficiently and effectively to the rest of the body.
Symptoms of pulmonary fibrosis can range from person to person, and can include:
- Shortness of breath (dyspnea)
- Rapid, shallow breathing
- Dry, hacking cough
- Gradual weight loss that is not intended
- Fatigue or tiredness
- Joint and muscle aches/pain
- "Clubbing" or widening of tips of fingers—this is caused by chronic lack of oxygen in the body, but it is not fully understood.
Because pulmonary fibrosis belongs to a family of related diseases, a thorough medical workup by a doctor is the only way to diagnose pulmonary fibrosis. The doctor will explore the patient’s medical and lifestyle history, and order tests such as x-rays and CT scans, breathing tests, lung biopsies, and blood tests. Sometimes, pulse oximetry, an echocardiogram (a heart test), arterial blood gas test, or exercise stress test may be done.
There is currently no cure for pulmonary fibrosis, though lung transplant may be an option for some individuals depending on their health. Treatments currently available cannot remove or repair scarring that has occurred in the lungs, and instead tries to prevent more scarring from developing. Clinical trials may help patients experience new treatment options and may provide better future treatments for pulmonary fibrosis.
Treatment for pulmonary fibrosis includes:
- Medicine:Most commonly, anti-inflammatory corticosteroids such as prednisone are used to treat the inflammation associated with pulmonary fibrosis scarring. Controversially, immune system suppressant medications are also used which can help prevent continued scarring which can extend the patient’s life—these are a matter of debate in the pulmonary medicine community. These medications do not work in all cases and may have serious side effects.
- Oxygen Therapy: Supplemental oxygen delivered by nasal cannula can deliver higher concentrations of oxygen to the lungs, helping patients breathe easier by compensating for scarred, damaged lungs. By providing adequate oxygen, work of breathing is reduced, thus reducing symptoms of shortness of breath and encouraging active living.
- Pulmonary Rehabilitation: Learning how to manage lung disease like pulmonary fibrosis is important to regain function for daily activities. Participants learn how to exercise, may learn about nutrition, and gain an understanding of their disease—they may also receive counseling or peer support.
- Lung transplant: For those under 65 in generally good health, lung transplantation can be considered, which can significantly improve quality of life.
Patients with pulmonary fibrosis can take an active role in staying healthy. In addition to the above, patients should take medications as directed by their doctor as well as adhering to all prescribed therapies and activities. Practicing other healthy behaviors such as getting a flu shot, quitting smoking and avoiding secondhand smoke, staying active, and consuming nutritious foods and a balanced diet can all help promote well-being, even with pulmonary fibrosis.
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Information on this page is for reference and educational purposes only. For more information about pulmonary fibrosis, talk to your doctor or primary care provider.
Page last updated: December 12, 2018