Spotlight On Interstitial Lung Diseases

Interstitial lung disease (ILD) is the name given to any disease that causes inflammation and/or scarring in the lungs.¹ ILD's are grouped based on what causes them. There are over 200 known causes of ILD's.²Treatment options depend on how much inflammation and scarring, also called fibrosis, your lungs have.²

How do my lungs normally work?

When you take a breath, oxygen moves into your lungs. Specifically, it moves into tiny sacs inside your lungs.³ These sacs are called alveoli.³ Their job is to push the oxygen from your lungs into your blood.³ Oxygen-rich blood is then pumped through your body to keep you alive.

How do ILD's impact my lungs?

ILD's make it hard for alveoli to push oxygen into the blood.⁴ This is because ILD's cause alveoli to swell or scar which causes the walls of the alveoli to thicken and stiffen.⁴ Thick alveoli are bad at moving oxygen out.⁴ Because of this, your body does not get fresh oxygen.

What are the symptoms of ILD's?

Stiff alveoli make the lungs less elastic meaning they cannot stretch.⁴ This causes the most common symptom, shortness of breath.² Some people have a dry cough and get tired easily.² Your symptoms may depend on which ILD you have.

What causes ILD's?

Some of the most common causes of ILD's are:^2,5-9

• Injury to the lungs – abnormal healing after an injury may cause alveolar scarring.
• Other diseases – autoimmune diseases like rheumatoid arthritis, lupus, sarcoidosis, and connective tissue diseases can lead to ILD's.
• Exposure – constantly breathing in chemicals can lead to ILD's. Silica and grain dust, asbestos, coal, tobacco, bird and animal droppings, salon chemicals, and radiation have all been shown to cause ILD's.
• Drugs – over 300 drugs, including chemotherapy and heart drugs, can cause ILD's.

Your ILD could be caused by a combination of these factors.⁶ Doctors also may not be able to tell you what caused your ILD, which would be called an idiopathic ILD.²

How are ILD's diagnosed?

Doctors will do an exam and ask for a detailed medical history.⁹ You may get a chest X-ray or a cat scan. You may see a specialist, called a pulmonologist, who will assess how well your lungs are working. Doctors may suggest a bronchoscopy or lung biopsy.⁶ These procedures look directly at your lungs to assess the damage.

What are the risk factors for ILD's?

Adults are more likely to get ILD's compared to children.⁷ Being around harmful chemicals, smoking, and radiation/chemotherapy all increase your risk.⁷ Gastroesophageal reflux disease (GERD) has also been linked to ILD's.^7,9

What is the treatment for ILD's?

Inflammation and fibrosis damage the lungs cannot be reversed.¹ Treating ILD's focuses on managing symptoms. Ways to manage symptoms include:^2,6,10-11

• Reduce inflammation – steroids can help lower inflammation. Drugs that lower your immune system response may work too.
• Manage fibrosis – anti-fibrotic drugs may slow the rate of lung scarring.
• Exercise your lungs – pulmonary rehabilitation keeps your lungs in shape. A respiratory therapist works with you to keep your lungs flexible so you can breathe with less shortness of breath.
• Oxygen therapy – your doctor may suggest that you wear oxygen at home to help oxygenate your body.
• Treat other symptoms – people with ILD's can have a nagging dry cough. You may be given drugs or cough medicine to ease your cough. GERD has been linked to ILD's so doctors may give you drugs to treat acid reflux.

Fibrosis complicates treatment for ILD's

ILD's can cause different amounts of inflammation or scarring. Doctors have described over 100 different ILD's.⁶ Each ILD causes varying amounts of swelling and fibrosis. Some ILD's have no fibrosis while some have a lot.⁶ ILD's with a lot of scarring are hard to treat.⁶

Idiopathic pulmonary fibrosis (IPF)

The most common ILD is idiopathic pulmonary fibrosis (IPF).^6,11 It is particularly hard to treat as it causes intense scarring.⁶ The average survival rate is anywhere from 2 to 5 years.¹¹

Two anti-fibrotic drugs, nintedanib and pirfenidone, are being tested to treat IPF.¹¹ Doctors are still trying to figure out how effective these drugs are for IPF. Some people in clinical trials had a slowing of IPF progression on these drugs.¹¹ Others showed that the drugs had no effect.¹¹ Many people with pulmonary fibrosis are put on a lung transplant list and encouraged to enroll in ILD clinical trials.⁶

What can I do to lower my risk of ILD's?

Get plenty of exercise, eat well, and do not smoke. Know your family history and manage any health issues you already have. Reduce your exposure to chemicals known to cause ILD's. If you must work with chemicals, try to open a window or get fresh air if possible.