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Pulmonary fibrosis is a serious disease that causes progressive scarring of your lung tissue. The current thinking is that pulmonary fibrosis begins with repeated injury to the tissue within and between the tiny air sacs (alveoli) in your lungs. The damage eventually leads to scarring (fibrosis), which stiffens your lungs and makes breathing difficult. The most common symptoms are shortness of breath and a dry cough. Current treatments for pulmonary fibrosis include medications and therapy to improve lung function and quality of life. A number of new therapies for pulmonary fibrosis are in clinical trials. In the meantime, a lung transplant may be an option for some people with pulmonary fibrosis.
Complications of Pulmonary Fibrosis
Causes of Pulmonary Fibrosis
Hundreds of factors can cause the lung damage that eventually leads to pulmonary fibrosis. Some of the most common include:
Idiopathic pulmonary fibrosis: When the cause isn't known
The list of substances and conditions that can lead to pulmonary fibrosis is long. Even so, in most cases, the cause is never found. Pulmonary fibrosis with no known cause is called idiopathic pulmonary fibrosis. Researchers have several theories about what might trigger idiopathic pulmonary fibrosis, including viruses and exposure to tobacco smoke. And because one type of idiopathic pulmonary fibrosis runs in families, heredity also is thought to play a role, even in people who don't directly inherit the disease.
Signs & Symptoms of Pulmonary Fibrosis
Signs and symptoms of pulmonary fibrosis include:
The most common pulmonary fibrosis symptoms are shortness of breath, especially during or after physical activity, and a dry cough. These often don't appear until the disease is advanced, and irreversible lung damage has already occurred. Even then, you may downplay your symptoms, attributing them to aging, being out of shape or the lingering effects of a cold. Breathing problems usually become progressively worse, and eventually you're likely to get out of breath during routine activities — getting dressed, talking on the phone, even eating. At this point, symptoms are impossible to ignore. The course of pulmonary fibrosis — and the severity of symptoms — can vary considerably from person to person. Some people become ill very quickly with severe disease. Others have more-moderate symptoms that grow worse over a period of months or years.
Diagnosis of Pulmonary Fibrosis
Diagnosing pulmonary fibrosis can be extremely challenging. The difference between idiopathic and nonidiopathic forms of the disease isn't always clear, and the naming and classification systems for both have historically been confusing and controversial. In addition, many medical conditions, including chronic obstructive pulmonary disease (COPD), asthma and even heart failure, can mimic pulmonary fibrosis, so doctors must rule these out before making a definitive diagnosis. A complete medical history, physical exam and even a chest X-ray aren't enough to diagnose pulmonary fibrosis, but they can help exclude other conditions. For that reason, you may have tests such as:
Often, though, pulmonary fibrosis can be definitively diagnosed only by examining a small amount of lung tissue (biopsy) in a laboratory. The tissue sample may be obtained in one of these ways:
Treatments of Pulmonary Fibrosis
The lung scarring that occurs in pulmonary fibrosis can't be reversed, and no current treatment has proved effective in stopping the ultimate progression of the disease. Some treatments, though, may improve symptoms temporarily or slow the disease's progress. Others help improve quality of life.
Many people diagnosed with pulmonary fibrosis are initially treated with a corticosteroid (prednisone), sometimes in combination with other drugs that suppress the immune system, such as methotrexate or cyclosporine. None of these combinations has proved very effective. Adding N-acetylcysteine, a derivative of a natural amino acid, to prednisone may slow the disease in some people. And in clinical trials, the drug pirfenidone has been shown to improve lung function and prevent destruction of lung tissue. Prednisone and other immunosuppressant medications can also cause serious side effects, including diabetes, glaucoma, reduced production of red blood cells, skin cancer and lymphoma. For that reason, treatment is usually discontinued if there's no improvement after six months. Although some people improve temporarily on immunosuppressant drugs, it's not clear why some people respond and others don't.
Lung transplantation may be an option of last resort for younger people with severe pulmonary fibrosis who haven't benefited from other treatment options. In order to be considered for a transplant, you must agree to quit smoking if you smoke, be healthy enough to undergo surgery and post-transplant treatments, be willing and able to follow the medical program outlined by the rehabilitation and transplant team, and have the patience and emotional strength and support to undergo the wait for a donor organ. The last requirement is particularly important because donor organs are in short supply.
Other treatment approaches
Other pulmonary fibrosis treatments focus on improving quality of life. They include:
Treatments under investigation
A number of treatments for pulmonary fibrosis are being developed or are in clinical trials. You can find an extensive listing of clinical trials in the National Institutes of Health clinical trial database on the Web. You can also contact the National Heart, Lung, and Blood Institute for more information. If you think you might be interested in participating in a clinical trial, your doctor can help you find an appropriate program.
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