Interstitial (in-tur-STISH-ul) lung disease describes a large group of disorders, most of which cause progressive scarring of lung tissue. The scarring associated with interstitial lung disease eventually affects your ability to breathe and get enough oxygen into your bloodstream.
Interstitial lung disease can be caused by long-term exposure to hazardous materials, such as asbestos. Some types of autoimmune diseases, such as rheumatoid arthritis, also can cause interstitial lung disease. In some cases, however, the causes remain unknown.
Once lung scarring occurs, it's generally irreversible. Medications may slow the damage of interstitial lung disease, but many people never regain full use of their lungs. Lung transplant is an option for some people who have interstitial lung disease.
The primary signs and symptoms of interstitial lung disease are:
By the time symptoms appear, irreversible lung damage has often already occurred. Nevertheless, it's important to see your doctor at the first sign of breathing problems. Many conditions other than interstitial lung disease can affect your lungs, and getting an early and accurate diagnosis is important for proper treatment.
Interstitial lung disease seems to occur when an injury to your lungs triggers an abnormal healing response. Ordinarily, your body generates just the right amount of tissue to repair damage. But in interstitial lung disease, the repair process goes awry and the tissue around the air sacs (alveoli) becomes scarred and thickened. This makes it more difficult for oxygen to pass into your bloodstream.
Interstitial lung disease can be triggered by many different things -- including airborne toxins in the workplace, drugs and some types of medical treatments. In most cases, the causes are unknown.
Long-term exposure to a number of toxins and pollutants can damage your lungs. These may include:
Some people who receive radiation therapy for lung or breast cancer show signs of lung damage months or sometimes years after the initial treatment.
Many drugs can damage your lungs, especially:
Lung damage can also result from autoimmune diseases such as:
The list of substances and conditions that can lead to interstitial lung disease is long. Even so, in some cases, the causes are never found. Disorders without a known cause are grouped together under the label of idiopathic interstitial pneumonias, the most common and deadly of which is idiopathic pulmonary fibrosis.
Factors that may make you more susceptible to interstitial lung disease include:
Interstitial lung disease can lead to a series of life-threatening complications, including:
Identifying and determining the cause of interstitial lung disease can be challenging. A large number of disorders fall into this broad category. In addition, the signs and symptoms of a wide range of medical conditions can mimic interstitial lung disease, and doctors must rule these out before making a definitive diagnosis.
Some of the following tests may be necessary.
Often, 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:
The lung scarring that occurs in interstitial lung disease can't be reversed, and treatment will not always be effective in stopping the ultimate progression of the disease. Some treatments may improve symptoms temporarily or slow the disease's progress. Others help improve quality of life.
Because many of the different types of scarring disorders have no approved or proven therapies, clinical studies may be an option to receive an experimental treatment.
Intense research to identify treatment options for specific types of interstitial lung disease is ongoing. Based on currently available, scientific evidence, however, your doctor may recommend:
Using oxygen can't stop lung damage, but it can:
You're most likely to receive oxygen when you sleep or exercise, although some people may use it round-the-clock.
The aim of pulmonary rehabilitation is not only to improve daily functioning but also to help people with intersitial lung disease live full, satisfying lives. To that end, pulmonary rehabilitation programs focus on:
Lung transplantation may be an option of last resort for some people with severe interstitial lung disease who haven't benefited from other treatment options.
Being actively involved in your own treatment and staying as healthy as possible are essential to living with interstitial lung disease. For that reason, it's important to:
Living with a chronic lung disease is emotionally and physically challenging. Your daily routines and activities may need to be adjusted, sometimes radically, as breathing problems worsen or health care needs take priority in your life. Feelings of fear, anger and sadness are normal as you grieve for the loss of your old life and worry about what's next for you and your family.
Share your feelings with your loved ones and your doctor. Talking openly may help you and your loved ones cope with the emotional challenges of your disease. In addition, clear communication will help you and your family plan effectively for your needs if your disease progresses.
You may also want to consider joining a support group, where you can talk to people who are facing challenges similar to yours. Group members may share coping strategies, exchange information about new treatments or simply listen as you express your feelings. If a group isn't for you, you may wish to talk with a counselor in a one-on-one setting.
You'll probably first bring your symptoms to the attention of your family doctor. He or she may refer you to a pulmonologist -- a doctor who specializes in lung disorders. Testing generally includes a variety of blood tests, a CT scan of the chest and pulmonary function testing.
Before your appointment, you might want to write a list that answers the following questions:
If your primary care physician had a chest X-ray done as part of your initial evaluation, bring that with you when you see a pulmonologist. It will help the pulmonologist make a diagnosis if he or she can compare an old chest X-ray with the results of a current X-ray.
The actual X-ray image is more important to your doctor than is the report alone. CT scans of your chest also may have been done, and those should also be requested.
Your doctor may ask some of the following questions:
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