Interstitial Lung Disease
Interstitial lung disease (ILD) is a group of disorders that leads to scarring, called fibrosis, in the lung tissue. It affects the space around the small air sacs of the lung.
All ILD disorders affect this particular area of the lung. However, the progression of diseases and the other parts of the lungs that may be affected are unique to each disorder.
Injury or illness can cause inflammation in the lungs and airways. Inflammation stimulates a process to rebuild injured tissue. With ILD, this inflammation and tissue building does not stop. Over time, the excess tissue building leads to fibrosis in the lungs. The fibrosis makes it difficult for oxygen to pass from the lung tissue to the blood vessels in the lungs. This decreases the amount of oxygen available to the body.
The inflammation and tissue building process may begin or go unchecked because of:
- Lung diseases or infections
- Long-term exposure to irritants such as cigarette smoke, dust, gasses, or fumes from industry or agriculture
- Autoimmune diseases
Certain medications, such as:
- Nonsteroidal anti-inflammatory drugs (NSAIDs)
- Medications that change or suppress the immune system
- Genetic abnormalities
Sometimes, the exact reason for the abnormal tissue building process is unknown.
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The risk of ILD increases with age. Other factors that may increase your chance of ILD include:
- Having a lung disease that causes fibrosis, such as idiopathic pulmonary fibrosis
- Having an autoimmune disorder, such as rheumatoid arthritis or scleroderma
- Working in occupations where you inhale dust, fibers, fumes, or bird droppings
- Family history or genetics
- Long-term medication use
- Cancer treatment—radiation and/or chemotherapy can damage lung tissue
The most common symptom of ILD is shortness of breath that worsens with time. Breathing problems occur with activity and at rest.
ILD may also cause:
- Persistent, dry cough that cannot be controlled
- Clubbing—nails that bulge or thicken
You will be asked about your symptoms and medical history. A physical exam will be done. ILD may be suspected based on this information.
To determine how well your lungs are working, your doctor may do the following tests:
- Blood tests
- Pulse oximetry—to measure the amount of oxygen in the blood
To confirm a diagnosis or determine the reason for scarring, a tissue sample may be removed from the lungs and closely examined. Images of the lungs and chest cavity may also be taken with:
If you are diagnosed with ILD, pulmonary function tests can help determine how much your breathing is affected.
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Treatment depends on the cause of ILD. The goal of treatment is to slow progression of the disease. Damage to the lungs is permanent and cannot be reversed, but treatment will help to ease symptoms and improve quality of life.
Treatment will be based on your specific condition and symptoms. General approaches include:
Medications can help to slow the progression of ILD. Options include:
- Corticosteroids to reduce inflammation
- Medications that suppress the immune system (interfere with the process of inflammation and fibrosis)
Oxygen therapy will eventually be needed. It will help to make up for decreased amount of oxygen passing to the bloodstream. Oxygen therapy increases the amount of oxygen in the lungs which increases the amount of oxygen that gets into your blood.
Pulmonary rehabilitation helps you manage shortness of breath. Rehabilitation is tailored to your needs but may include:
- Breathing techniques and coping strategies
- Support from others
Learning to live with a chronic disease can be a difficult process. You may benefit from psychological counseling that can help you better manage your life. Counseling can be individual or in a group.
If you smoke, your doctor will advise you about the most effective programs to help you quit.
A lung transplant may be necessary if ILD has progressed or is advanced. It is generally not considered unless other treatment methods fail.
Not all ILDs can be prevented. To help reduce your exposure to substances associated with some ILDs:
- Talk to your doctor about ways to help you quit smoking.
- Avoid lung irritants whenever possible.
- Follow occupational guidelines to protect your lungs at work.
- Use proper protection when exposed to harmful chemicals, dust, or animal droppings.
American Lung Association
National Heart, Lung, and Blood Institute
Canadian Pulmonary Fibrosis Foundation
The Lung Association
Explore pulmonary rehabilitation. National Heart, Lung, and Blood Institute website. Available at: http://www.nhlbi.nih.gov/health/health-topics/topics/pulreh. Accessed March 14, 2018.
Interstitial lung disease. EBSCO DynaMed Plus website. Available at:http://www.dynamed.com/topics/dmp~AN~T900225/Interstitial-lung-disease. Updated January 23, 2018. Accessed March 14, 2018.
Interstitial lung disease (ILD). British Lung Foundation website. Available at: https://www.blf.org.uk/support-for-you/interstitial-lung-disease-ild. Accessed March 14, 2018.
Overview of interstitial lung disease. Merck Manual Professional Version website. Available at: http://www.merckmanuals.com/professional/pulmonary-disorders/interstitial-lung-diseases/overview-of-interstitial-lung-disease. Updated April 2016. Accessed March 14, 2018.
Schraufnagel DE. Chapter 10: Interstitial lung disease. American Thoracic Society website. Available at: https://www.thoracic.org/patients/patient-resources/breathing-in-america/resources/chapter-10-interstitial-lung-disease.pdf. Accessed March 14, 2018.
Last reviewed March 2018 by EBSCO Medical Review Board James P. Cornell, MD Last Updated: 3/8/2016