Reviewed by Gregory P. Cosgrove, MD, FCCP, Jeffrey James Swigris, DO, MS, Tristan J. Huie, MD
Treatment for ILD is based upon the diagnosis and may differ depending on the diagnosis.

Many forms of ILD can be treated successfully with medications. ILD that is primarily the result of inflammation in the lung can often be treated with immunosuppressive medications. Idiopathic pulmonary fibrosis (IPF) does not respond to immunosuppressive medications, but two medications have recently been approved by the FDA for the treatment of IPF. They are pirfenidone and nintedamib.

However, our current available therapies are not thought to reverse scarring or fibrosis that has already taken place. We are constantly researching new medications that may work to halt or reverse scarring. In some cases where medication therapy is not felt to be useful, a lung transplant is recommended. Because ILD can result in respiratory failure, it is important to diagnose and treat it as early as possible.

If your doctor has identified an occupational or environmental exposure, removal from the source of the problem is essential-even if it means giving up your hobby or pet, or changing what you do in your job or where you live.

Regardless of the cause of ILD, the goals of treatment are:

  • To decrease inflammation and prevent further lung scarring,

  • To remove the source of the problem, when possible,

  • To minimize and manage potential complications of ILD and

  • To improve or prevent deterioration in a person's quality of life.



Many medications are used to treat interstitial lung disease and its various symptoms. The medications seek to make breathing more productive, reduce inflammation, and suppress overactive immune systems. Learn about medications to treat ILD.


Oxygen Therapy

Oxygen is required for some people with ILD because of low level of oxygen in the blood. Some may need oxygen therapy all of the time while others may need it only during sleep and exercise. Improving the level of oxygen in the blood through the use of supplemental oxygen can help relieve the strain on the heart and lungs and improve symptoms of shortness of breath and fatigue. There is a stigma often associated with oxygen. Many people are embarrassed and are concerned with how it will look and worry about how it will change their lifestyle. There are different options for oxygen systems and people are still able to get out their homes and even travel. Most people find once they are on oxygen they are able to be more active as they are not as short of breath. National Jewish Health can discuss what oxygen system will best fit your lifestyle.


Pulmonary Rehabilitation

A pulmonary rehabilitation program is recommended to help you achieve your highest level of functioning. This program includes education, exercise conditioning, breathing techniques, and energy saving techniques, respiratory therapy evaluation, nutritional counseling and psychosocial support.

The specific goals of pulmonary rehabilitation are to improve quality of life by: decreasing respiratory symptoms and complications, encouraging self-management and control over daily functioning, improving physical conditioning and exercise performance, improving emotional well-being and reducing hospitalizations.


Lung Transplant

If other therapies fail to adequately treat ILD, lung transplant is an option for some advanced cases. With improved surgical techniques and post-transplant care, this may offer improved quality of life and prolonged survival to selected patients.

Response to therapy varies widely. Some types of ILD may respond quickly and others may not respond at all. Treatment is considered successful if symptoms, physiological, and x-ray findings are stabilized. Even with treatment, many types of ILD progress naturally with a worsening of symptoms, x-ray, and physiologic findings. Sometimes worsening is due to a complication of the disease or therapy. This may include conditions such as pulmonary hypertension or right heart failure. Some therapies may result in infection, muscle weakness and osteoporosis.

For transplant centers nearest to you, visit the Organ Procurement and Transplantation Network website.

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