Interstitial (in-tur-STISH-ul) lung disease, also called ILD, describes a large group of conditions. Most of these conditions cause inflammation and progressive scarring of lung tissue. As part of this process, lung tissue thickens and stiffens, making it hard for the lungs to expand and fill with air.

At some point, the scarring from interstitial lung disease makes it harder to breathe and get enough oxygen into the bloodstream. Many people with ILD are short of breath with activity and may have a bothersome dry cough.

Interstitial lung disease can have many causes, including long-term exposure to hazardous materials such as asbestos. Some types of autoimmune diseases, such as rheumatoid arthritis, also can cause interstitial lung disease. But the cause isn't known sometimes. ILD can have many causes, so treatment varies.

The disease may get worse slowly or rapidly at a pace that often can't be predicted. Once lung scarring occurs, it generally isn't reversible. Treatment focuses on keeping more scarring from occurring, managing symptoms and making quality of life better. Medicines may slow the damage of interstitial lung disease, but many people never fully use their lungs again. Lung transplant is an option for some people who have ILD.

The main symptoms of interstitial lung disease are:

• Shortness of breath at rest or shortness of breath that worsens with physical activity.
• Dry cough.

When to see a doctor

By the time symptoms appear in certain types of interstitial lung disease, lasting lung damage has already occurred. That's why it's important to see your healthcare professional at the first sign of breathing problems. Many conditions other than ILD can affect your lungs. Getting an early and correct diagnosis is important for proper treatment.

To prevent interstitial lung disease, avoid exposure to toxins at work, such as asbestos, coal dust and silica dust. Also, avoid exposure to toxins in the environment, such as bird protein, mold and bacteria. If you must be around these toxins, protect yourself by wearing a respirator. Other ways to prevent ILD include not smoking and avoiding secondhand smoke.

If you have an autoimmune disease or are taking medicines that raise your risk of getting ILD, talk with your healthcare professional about steps you can take to prevent ILD. Also, get vaccinated because respiratory infections can make symptoms of ILD worse. Be sure you get the pneumonia vaccine and a flu shot each year. Also, ask your healthcare professional about getting vaccinated for pertussis, COVID-19 and respiratory syncytial virus, also called RSV.

Factors that may make you more likely to get interstitial lung disease include:

• Age. ILD is much more likely to affect adults, although babies and children sometimes get the disease.
• Exposure to toxins at work or in the environment. Working in mining, farming or construction, or for any reason getting exposed to pollutants known to damage lungs, raises your risk of getting ILD.
• Smoking. Some forms of ILD are more likely to occur in people with a history of smoking. Active smoking may make the condition worse, especially if you also have emphysema.
• Radiation and chemotherapy. Having radiation treatments to your chest or using certain chemotherapy drugs raises your risk of lung disease.
• Connective tissue disease. This includes autoimmune diseases that can raise your risk of ILD.

Interstitial lung disease can lead to a series of life-threatening complications, including:

• High blood pressure in your lungs, also known as pulmonary hypertension. Unlike systemic high blood pressure, this condition affects only the arteries in your lungs. Scar tissue or low oxygen levels restrict the smallest blood vessels, limiting blood flow in your lungs. This raises pressure within the pulmonary arteries and can worsen oxygen exchange, lowering oxygen levels in your blood. Pulmonary hypertension is a serious illness that may get worse over time, causing the right side of your heart to fail.
• Right-sided heart failure, also known as cor pulmonale. This serious condition occurs when your heart's lower right chamber, also known as the right ventricle, must pump harder than usual to move blood through blocked pulmonary arteries. Eventually, the right ventricle fails from the extra strain. This is often due to pulmonary hypertension.
• Respiratory failure. In the end stage of chronic ILD, respiratory failure occurs when severely low blood oxygen levels, along with rising pressures in the pulmonary arteries and the right ventricle, cause the heart to fail.

Lung scarring that already has occurred in interstitial lung disease can't be reversed, and treatment won't always stop the disease from getting worse. Some treatments may make symptoms better for a short time or slow the disease. Others help maintain quality of life.

Because many of the different types of scarring diseases have no approved or proven therapies, clinical trials may be an option to get an experimental treatment.

Medications

Intense research to find treatment options for specific types of interstitial lung disease is ongoing. Treatment may vary depending on the cause of ILD and what damage has happened in the lungs. Using on the latest scientific evidence, your healthcare professional may recommend:

• Corticosteroid medicines. At first, many people diagnosed with ILD are treated with a corticosteroid, namely prednisone (Prednisone Intensol, Rayos). Sometimes people are treated with other drugs that suppress the immune system. Depending on the cause of ILD, these medicines may slow or even keep the disease from getting worse.
• Medicines that slow the worsening of idiopathic pulmonary fibrosis. Pirfenidone (Esbriet) and nintedanib (Ofev) are medicines that may slow the rate at which IPF worsens. Ofev also has been approved for people with lung fibrosis that's getting worse due to other types of interstitial lung disease. Side effects for both drugs are common. Talk with your healthcare professional about the pros and cons of these medicines.
• Medicines that reduce stomach acid. Gastroesophageal reflux disease, also known as GERD, affects most people with idiopathic pulmonary fibrosis. GERD is linked to worse lung damage. If you have symptoms of acid reflux, your healthcare professional may prescribe ways to treat GERD that reduce stomach acid.

Oxygen therapy

Using oxygen can't stop lung damage, but it can:

• Make it easier to breathe and exercise.
• Prevent or lessen complications from low blood oxygen levels.
• Lower blood pressure in the right side of your heart.
• Make your sleep and sense of well-being better.

You're most likely to get oxygen when you sleep or exercise, although some people may use it around the clock.

Pulmonary rehabilitation

The aim of pulmonary rehabilitation is to make you better able to function and live a full, satisfying life. That's why pulmonary rehabilitation programs focus on:

• Learning more about your lung disease.
• Exercise, so you can become more physically active for longer periods of time.
• Breathing techniques that make your lungs more efficient.
• Emotional support.
• Nutritional counseling.

Surgery

A lung transplant may be an option of last resort for some people with severe interstitial lung disease when other treatment options haven't helped.

You must be actively involved in your own treatment and stay as healthy as possible when you're living with interstitial lung disease. For that reason, it's important to:

• Learn about your disease. Understanding your condition and how it can be treated can help you decide about your care. Including family members and friends can help them learn your needs.
• Stop smoking. If you have lung disease, the best thing you can do for yourself is to stop smoking. Talk with your healthcare professional about options for quitting, including programs to help you stop smoking. These programs use various proven techniques to help people quit. And because secondhand smoke also can harm your lungs, don't allow people to smoke around you.
• Avoid exposure at work or during hobbies. When possible, stay away from substances that can irritate your lungs. Ask your healthcare professional for more information and advice.
• Eat well. If you have lung disease, you may lose weight because it isn't comfortable to eat and because of the extra energy it takes to breathe. Aim to eat a nutritionally rich diet that contains enough calories. A dietitian can give you more guidelines for healthy eating.
• Get vaccinated. Respiratory infections can make symptoms of ILD worse. Make sure you get the pneumonia vaccine and a flu shot each year.

Living with a chronic lung disease is emotionally and physically challenging. You may need to change your daily routines and activities — sometimes a lot — as breathing problems worsen or health care needs become more important. Feelings of fear, anger and sadness are typical as you grieve for the loss of your old lifestyle and worry about what's next for you and your family.

Share your feelings with your loved ones and your healthcare professional. Talking openly may help you and your loved ones cope with the emotional challenges of your disease. Also, clear communication can help you and your family plan for your needs if your disease gets worse.

Think about joining a support group, where you can talk with people who are facing challenges like yours. Group members may share coping strategies, exchange information about new treatments or simply listen as you express your feelings. If a group isn't for you, you may want to talk with a counselor in a one-on-one setting.

You'll probably first see your family healthcare professional about your symptoms. Your family healthcare professional may refer you to a pulmonologist, who is a doctor who specializes in lung diseases.

What you can do

Before your appointment, you might want to prepare answers to these questions:

• What are your symptoms and when did they start?
• Are you being treated for other medical conditions?
• What medicines and supplements have you taken in the past five years, including medicines available without a prescription and street drugs?
• What are all the occupations you've had, even if only for a few months?
• Do any members of your family have a lasting lung disease of any kind?
• Have you ever had chemotherapy or radiation treatments for cancer?

Let your pulmonologist know if you've had any chest X-rays, CT scans or other tests so the healthcare professional can request the results before your visit. The images are more important than the report alone. The pulmonologist can make a diagnosis by comparing an old imaging test with those of a current test.

What to expect from your doctor

Your doctor or other healthcare professional may ask some of these questions:

• Do your symptoms occur all of the time or do they seem to go away and then come back?
• Have you recently had contact with air conditioners, humidifiers, pools, hot tubs, or water-damaged walls or carpet?
• Do you own any pet birds or feather-containing items such as down pillows or comforters?
• Are you exposed to mold or construction dust in your home or other places where you spend a lot of time?
• Have any close relatives, friends or co-workers been diagnosed with a lung condition?
• Do you have any family history of lung disease?
• Does your work history include regularly working with or being around toxins and pollutants, such as asbestos, silica dust or grain dust?
• Do you or did you smoke? If so, how much and for how long? If not, have you spent a lot of time around others who smoke?
• Have you been diagnosed or treated for any other medical conditions, particularly arthritis or rheumatic diseases?
• Do you have symptoms of gastroesophageal reflux disease, also known as GERD, such as heartburn?