Interstitial Lung Disease

There are many types of interstitial lung disease — about 200 to be exact. Some of these are genetic (inherited). Some start for no apparent reason (unknown or idiopathic). Others are caused by an illness or other conditions, including connective tissue diseases. Tobacco use, medication, radiation therapy or inhaling harmful environmental or occupational toxins — like asbestos or silicates — can also trigger different kinds of interstitial lung disease.

Some of the ILD types we treat at Cleveland Clinic include:

Idiopathic lung disease

• Idiopathic pulmonary fibrosis.
• Familial (genetic) pulmonary fibrosis.
• Nonspecific interstitial pneumonia.
• Cryptogenic organizing pneumonia.
• Desquamative interstitial pneumonia.
• Respiratory bronchiolitis-associated ILD.
• Pulmonary Langerhans cell histiocytosis.

Sarcoidosis-related lung disease

• Pulmonary sarcoidosis.
• Cardiac sarcoidosis.

Autoimmune/connective tissue-related lung disease

• Scleroderma.
• Rheumatoid arthritis.
• Dermatomyositis/Polymyositis.
• Systemic lupus erythematosus (lupus).
• Granulomatosis with polyangiitis (GPA, formerly called Wegener’s).
• Eosinophilic granulomatosis with polyangiitis (EGPA, formerly called Churg-Strauss syndrome).
• Microscopic polyangiitis (MPA).

Occupational/exposure-related lung disease

• Hypersensitivity pneumonitis.
• Silicosis.
• Beryllium disease (berylliosis).
• Asbestosis.

Rare lung diseases

• Pulmonary alveolar proteinosis (PAP).
• Lymphangioleiomyomatosis (LAM).

All types of ILD cause inflammation (swelling) and/or scarring in your lungs — specifically the small air sacs called alveoli and the blood vessels surrounding them. This can make it harder for your body to move oxygen from your lungs into the rest of your body, which can leave you feeling short of breath and tired. And you may have a dry cough or feel a tightness in your chest.

If you have other conditions like heart disease or an autoimmune disease, this can worsen your symptoms. Often, ILD causes irreversible damage.

At your first appointment, your providers will go over your health history with you to see if any other conditions may be contributing to your ILD. You’ll also have a physical exam, where we’ll listen to your lungs, check out your overall health and maybe do some blood work to see if your blood shows any signs of an ILD. And we may recommend some other tests that can help us get a better idea of what’s going on inside of your chest.

Pulmonary function tests

We use pulmonary function tests (PFT) to get a better idea of how well your lungs work. These painless, noninvasive tests let us see how much air goes in and out of your lungs, how much air moves from your lungs to your blood and how they work when you’re exercising. There are different types of PFTs, including:

• Spirometry.
• Body plethysmography (lung volumes).
• Gas diffusion study.
• Cardiopulmonary exercise test (CPET).

Imaging tests

Tests like X-rays or a high-resolution CT scan let us to take pictures of your lungs to look for problems like fibrosis (scarring).

Bronchoscopy

A bronchoscopy gives us a close look into your lung’s airways. We do this by inserting a thin, flexible tube with a camera on it (bronchoscope) through your nose or mouth and moving it into your lungs. You’ll be sedated for this minimally invasive procedure, so you’ll be drowsy and won’t feel any discomfort.

Biopsy

During your bronchoscopy, your provider may also do a biopsy. They’ll remove a small sample of tissue from your lung and send it to the lab. Our pathologists will look at it under a microscope to help find out what type of interstitial lung disease you may have.

Sometimes, we might do a biopsy without a bronchoscopy. If we do, you’ll have general anesthesia that puts you to sleep so you won’t feel any pain. We’ll make several small incisions (cuts) on your chest between your ribs and carefully remove tissue samples from the incisions.

Meet Our Interstitial Lung Disease Care Team

Our skilled pulmonologists lead this team, which (depending on your needs) could also include:

• Rheumatologists.
• Thoracic radiologists who specialize in lung imaging.
• Pulmonary pathologists who specialize in testing lung tissues.
• Thoracic surgeons.
• Cardiologists.
• Gastroenterologists.
• Physical therapists.
• Respiratory therapists.
• ILD specific nurse practitioners and physician assistants.
• ILD specific nurses.

While we can’t cure interstitial lung disease, we can help you manage it by improving your symptoms and quality of life. Once you finish testing, and your care team reviews the results, they’ll sit down with you to talk about what treatment options they think will work for you.

You’ll have a highly personalized treatment plan that considers what’s causing your interstitial lung disease, how badly your symptoms affect your life and your overall health and lifestyle.

You may take medication to reduce inflammation and disease progression and slow down scarring. You might need extra oxygen to help you breathe easier. And we might decide you should do physical therapy to help you stay strong and mobile.

Lifestyle changes

If environmental exposure has played a role in why you have interstitial lung disease, you may need to change jobs. If you smoke, you may need to quit smoking. Your provider may connect you with a social worker to help you navigate these new changes in your life.

Another health condition may cause your ILD to get worse. We’ll refer you to a specialist to help you manage related conditions, like autoimmune disorders, heart disease, gastroesophageal reflux disease (GERD) and connective tissue disease.

Medication

We use several types of medication to treat interstitial lung disease:

• Corticosteroids to reduce inflammation.
• Antifibrotic drugs to slow down lung scarring.
• Biologic drugs to treat any autoimmune diseases and other causes of ILD.
• Antacids or proton pump inhibitors (PPIs) to treat GERD by reducing stomach acid.

We’ll make sure you understand your medication’s potential side effects and help you adjust your dosage, if needed.

Pulmonary rehabilitation

Our respiratory and physical therapists can help improve your quality of life through pulmonary rehabilitation. They help you strengthen your lungs through exercise and self-care. With consistency, pulmonary rehab can reduce your shortness of breath and improve your ability to do daily activities.

Oxygen therapy

If we discover your lungs aren’t providing enough blood and oxygen to your blood and tissues, we may prescribe extra oxygen for you. You’ll get the oxygen through a mask or a tube in your nose.

Lung transplant

If ILD’s severely damaged your lungs and other treatments don’t help, your providers may explore the possibility of a lung transplant. With this option, you’d go on a waiting list to get a donor lung to replace your damaged one — and have surgery when a lung is available. Our lung transplant program is among the nation’s largest and most experienced programs, giving you the best chance for success. You’ll have a team of experienced, caring providers guiding you through the transplant process so you understand what to expect at each step — from your first appointment until after you’ve recovered.

Taking the Next Step

Having trouble breathing can be scary. So can not knowing what to do next. Cleveland Clinic is here to take the guesswork out of things — and help you take the next step. Our healthcare providers treat many types of interstitial lung disease and can craft a personalized treatment plan that helps you manage your symptoms so you can breathe (and feel) better.