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.

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’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 ?

Shortness of breath (Dyspnea) that gets worse with exercise or exertion.

Dry cough.
Difficulty in breathing.
Fatigue
Chest discomfort.
Unexplained weight loss
Widening and rounding of the tips of the fingers or toes (clubbing)
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.

What causes interstitial lung disease ?

Connective tissue disease, including rheumatoid arthritis (RA), lupus and scleroderma.
Granulomatous disease, like sarcoidosis.
Inhaling certain substances, like asbestos, silica, tobacco and beryllium.
Allergic reaction to something you inhale(hypersensitivity pneumonitis), including molds, fungi, bacteria and bits of bird feathers or droppings.
Medications or treatments, including amiodarone, nitrofurantoin, methotrexate and radiation therapy.
When the cause of interstitial lung disease can't be determined, it’s called idiopathic interstitial pneumonia.
The most common ILD with an unknown cause is idiopathic pulmonary fibrosis.

How is interstitial lung disease diagnosed ?

Pulmonary function tests. Pulmonary function tests are usually breathing tests, but they can also be blood tests or exercise tests. They check how well your lungs are working.

Imaging tests. X-rays or high-resolution CT scans can be used to get pictures of your lungs.

Blood tests. Your provider will test a sample of your blood, taken from your arm, for signs of known causes of ILD.

Bronchoscopy. Your provider will insert a thin tube (bronchoscope) through your nose or mouth into your lungs to look at your airways.

Biopsy. Your provider will remove a small sample of tissue from your lung and study it under a microscope to help identify the type 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 ?

Corticosteroids: They can help reduce inflammation.
Anti-fibrotic and cytotoxic drugs: These medications can slow down lung scarring.
Biologic drugs: These medications are sometimes used to treat autoimmune diseases and other causes of ILD.
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 ?

Avoid or wear a respirator (a mask that filters particles from the air) 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.

What are the complications of interstitial lung disease ?

High blood pressure in your lungs (pulmonary hypertension).
Collapsed lung (pneumothorax).
Lung infections.
Respiratory failure.
Lung cancer.

What is the life expectancy of a person with interstitial lung disease ?

The life expectancy for interstitial lung disease depends on the cause and severity. Some people with mild ILD can live normal lives without treatment or with minimal treatment. Your outlook (prognosis) is better if your disease isn’t getting worse (it’s stable). For people with the most severe and rapidly worsening forms of interstitial lung disease, life expectancy is around 3-5 years after diagnosis.

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.