Interstitial Lung Disease

Interstitial lung disease encompasses a group of disorders that
cause progressive scarring of lung tissue.

Interstitial Lung Disease

What is interstitial lung disease?

Interstitial lung disease (ILD) is a term for a group of over 200 conditions that cause inflammation and scarring in your lungs. ILD damages the tissues between the small air sacs in your lungs (alveoli) and the blood vessels around them. This makes it harder for you to move oxygen out of your lungs and into your body.

Interstitial lung disease is also called diffuse parenchymal lung disease (DPLD).

What happens when you have interstitial lung disease?

When you have interstitial lung disease, parts of your lungs that help oxygen get into your blood and out to your tissues are damaged. Scarring in your lungs makes it hard to breathe, and you might have a chronic cough. Lack of oxygen can make you feel tired all the time.

When damage continues to get worse, you can have life-threatening complications, like lung infections and respiratory failure (not enough oxygen or too much carbon dioxide in your body).

What are examples of interstitial lung disease?

There are over 200 interstitial lung diseases. The most common is idiopathic pulmonary fibrosis, followed by diseases caused by occupational exposures or connective tissue disorders. A few other examples include:

Asbestosis.
Silicosis.
Radiation pneumonitis.
Nonspecific interstitial pneumonia.

What’s the difference between interstitial lung disease and pulmonary fibrosis?

Pulmonary fibrosis is a type of interstitial lung disease. Not all ILD is pulmonary fibrosis.

Who is most at risk for interstitial lung disease?

You’re more likely to develop interstitial lung disease if you:

Are over 70.
Were assigned male at birth.
Smoke or used to smoke.
Have a history of certain illnesses or conditions, like hepatitis C, tuberculosis, pneumonia, COPD or connective tissue disease.
Work around substances that can irritate your lungs, like asbestos, silica, molds, fungi or bacteria.
Have had chest radiation.

How serious is interstitial lung disease?

Since there are many kinds of interstitial lung diseases, any one case could be mild or very serious. Most kinds of ILD cause irreversible lung damage. The most serious ILDs are progressive, where your condition continues to worsen over time.

What are the symptoms of interstitial lung disease?

Common symptoms of interstitial lung disease include:

Shortness of breath (dyspnea) that gets worse with exercise or exertion.
Dry cough.
Fatigue.
Chest discomfort.

Symptoms are usually mild at first but get worse over months or years. You may have additional symptoms depending on the underlying cause of ILD.

How is interstitial lung disease treated?

There’s no cure for interstitial lung disease. Treatment for ILD usually focuses on treating underlying disease and improving your symptoms. Your healthcare provider might prescribe physical therapy, supplemental oxygen or medication to reduce inflammation or slow down the disease.

What medications and treatments are used in interstitial lung disease?

Anti-fibrotic and cytotoxic drugs. These medications can slow down lung scarring. They include azathioprine, cyclophosphamide, pirfenidone, and nintedanib.
Biologic drugs. Medications like rituximab are sometimes used to treat autoimmune diseases and other causes of ILD.
Treatment for GERD. Gastroesophageal reflux disease (GERD) can make ILD worse, so your provider may prescribe medications to keep stomach acid down.
Pulmonary rehabilitation. Breathing exercises and physical therapy can make your lungs stronger and breathing easier.
Oxygen therapy. Your provider will prescribe extra oxygen if you don’t have enough getting to your blood or tissues. It’s delivered through a mask or tube in your nose.
Lung transplant. Some people with severe cases of ILD get a lung transplant.

How can I prevent interstitial lung disease?

Many causes of interstitial lung disease aren’t preventable. You can reduce your risk of ILD by managing underlying conditions and avoiding breathing in harmful substances.

Avoid or wear a respirator when working around harmful substances, such as asbestos, metal dusts, or chemicals.
Avoid or wear a respirator when working around things that can cause chronic allergic reactions, like hay, grain, bird droppings, or feathers and heating and cooling systems.
If you have a connective tissue disease or sarcoidosis, talk to your healthcare provider about ways to manage your illness to prevent ILD.
Don’t smoke or quit smoking.

What can I expect if I have interstitial lung disease?

How you and your healthcare provider manage interstitial lung disease depends on the cause. If you have an occupational exposure or allergy (hypersensitivity pneumonitis), you may have to stop working with whatever’s causing your ILD or take extra precautions.

While medications can help bring down inflammation, scarring caused by interstitial lung disease is permanent. If you have an underlying disease, management of it might help prevent further damage. If the cause is unknown, your healthcare provider will treat your symptoms and try to prevent more damage.

When should I see my healthcare provider?

See your healthcare provider if you’ve had increasing shortness of breath, shortness of breath with exercise, or an ongoing dry cough. The outlook for interstitial lung disease is best if treated as soon as possible.

What questions should I ask my healthcare provider?

What’s causing my disease?
What are my treatment options?
Will it get worse?
Can the damage be slowed down?
What changes can I make to improve my quality of life?

Interstitial Lung Disease