Interstitial Lung Disease & Pulmonary Fibrosis

Interstitial lung disease (ILD) refers to more than 200 chronic lung disorders, including pulmonary fibrosis. With ILD, the tissue between the air sacs of the lungs (the interstitium) is affected by inflammation or scarring (fibrosis).

These disorders generally are:

• Chronic (lifelong)
• Non-malignant (not directly cancerous)
• Non-infectious (you can't pass the disease to another person)

Fibrosis causes lung tissue to permanently lose the ability to breathe and carry oxygen. Air sacs, as well as the lung tissue between and surrounding the air sacs, and the lung's small blood vessels (capillaries), are destroyed by the formation of scar tissue.

Physicians don't know the cause of many common types of ILD, including the most common variety known as idiopathic pulmonary fibrosis. In other cases, ILD has been shown to be caused by one of the following:

• Inhaling environmental pollutants

• Occupational hazards, such as prolonged exposure to asbestos fibers, can cause inflammation and lead to the scarring in the lung.

• Widespread diseases that "attack" the body, such as rheumatoid arthritis, can lead to a variety of interstitial lung diseases.

The symptoms will vary from person to person, but the most common symptoms of an interstitial lung disease are:

• Shortness of breath, especially with exertion or exercise

• Dry cough that does not produce phlegm

• Labored breathing

Although rapid worsening can happen, interstitial lung disease symptoms generally progress slowly and gradually get worse over time. Because these symptoms may resemble other lung conditions or medical problems, accurate diagnosis is essential to ensure proper treatment.

Usually a physician will be able to diagnose interstitial lung disease using a chest X-ray or lung CT scan. But some people with ILD have a normal chest X-ray. In many cases, your doctor may ask for one or more of the following tests:

• Pulmonary function tests (PFT): You will be asked to blow into a special machine called a spirometer, which measures whether or not you have decreased lung capacity. This test is also used to monitor ILD.

• Exercise testing: During this test, your doctor will ask you to either ride a stationary bike or walk on a treadmill while he or she monitors your breathing and blood oxygen level.

• Bronchoscopy: For this test, you will be awake but sedated. Your doctor will pass a flexible tube through your mouth and into your lungs. He or she will then be able to remove a very small sample of lung tissue for testing. The procedure can usually be completed in your doctor's office.

• Open lung biopsy: Done in rare cases, an open lung biopsy requires a patient to have surgery under general anesthesia. A surgeon will make a small incision between two ribs in order to remove a small sample of lung tissue. Like most open surgeries, you will probably have to spend time in the hospital.

• Bronchoalveolar lavage (BAL): BAL is similar to a bronchoscopy except instead of removing lung tissue, your doctor will inject and then immediately remove saline solution from your lungs. This procedure is frequently used to determine whether or not your ILD is active or non-active.

The course of interstitial lung disease is unpredictable. Because each patient is different, your ILD may run a gradual course or a rapid course. You may notice a variation in symptoms, from very mild, to moderate, to very severe. The condition may remain the same for long periods of time or it may change quickly. At UChicago Medicine, your physician will closely monitor your condition for change and adjust your treatment as needed.

If your ILD progresses, the lung tissue thickens and becomes stiff. Breathing then becomes more difficult and demanding. Some of the diseases improve with medication if treated when inflammation occurs. Some people may need oxygen therapy as part of their treatment.

Interstitial lung disease patients and pulmonary fibrosis patients may be candidates for a lung transplant. If you are a candidate, our experts will help you through the lung transplant process.