Interstitial Lung Disease (ILD)

Interstitial lung disease (ILD) is a broad term that refers to more than 200 different lung diseases. These conditions have a variety of causes and consequences, as well as a wide range of symptoms from minimal shortness of breath to life-threatening disability due to progressive inflammation and or scarring (fibrosis) of your lung tissue.

ILD can damage the small air sacs in your lungs (alveoli), the glue that hold the lungs together (interstitium), your airways, and/or the surrounding blood vessels. That means your lungs must work harder to push oxygen to the rest of your body, making breathing more difficult.

Some ILDs only affect the lungs, while other ILDs like sarcoidosis can affect any organ system. Sarcoidosis leads to deposits of small clumps of immune cells (granulomas) in tissue. These clumps can damage lungs, heart, skin, eyes, or any other affected organ due to inflammation and/or scarring.

While idiopathic pulmonary fibrosis (IPF) is a common cause of ILD, it is important to be seen by an ILD provider to thoroughly evaluate for and exclude other causes of ILD because treatment and consequences of the disease may differ based on the specific type of ILD you have.

Causes of ILD that can be initially and mistakenly diagnosed as IPF include:

• Hypersensitivity pneumonitis 
• Connective tissue disease-associated ILD 
• Drug-induced ILD 
• Sarcoidosis 
• Aspiration

Early signs of ILD are usually mild. However, they get worse over time. The most common ILD symptoms include:

• Chest discomfort
• Dry cough
• Fatigue
• Shortness of breath (at rest or with physical activity)

Early diagnosis is critical with ILD. By the time you experience symptoms, you already have lung damage. So, make an appointment with us as soon as your breathing problems start.

There are known and unknown (idiopathic) causes of ILD.

Some of the known ILD causes include:

• Hypersensitivity reactions to something you regularly breathe in (hypersensitivity pneumonitis)
• Connective tissue diseases like lupus, rheumatoid arthritis or scleroderma
• Granulomatous diseases like sarcoidosis
• Inhaling inorganic dust particles that deposit in and irritate your lungs (pneumoconiosis)
• Some medications or treatments like amiodarone, methotrexate, radiation therapy and some antibiotics

You are at higher risk for an ILD condition if you:

• Are a current or former smoker
• Are over age 70
• Have a family history of ILD
• Have had chest radiation
• Have a history of certain conditions, including chronic obstructive pulmonary disorder (COPD), connective tissue disease, hepatitis C, pneumonia or tuberculosis
• Were assigned male at birth
• Work around lung-irritating substances like asbestos, bacteria, fungi, mold or silica

Our experts will complete a thorough evaluation to diagnose your ILD. During a physical exam, they will:

• Ask about any activities that could increase your ILD risk
• Evaluate your personal and family medical histories
• Listen to your lungs
• Review your medications

If our providers need additional information, they may suggest these tests:

• Blood tests: Based on your history and exam, specific blood tests may be ordered to look for conditions like connective tissue disease, sarcoidosis, and hypersensitivity pneumonitis
• Bronchoscopy: Our pulmonologists thread a thin bronchoscope tube through your mouth or nose to your lungs to examine your airways
• Imaging tests: Our pulmonologists may request a CT scan or X-ray to examine your lungs
• Pulmonary function tests: Our experts may use breathing or exercise tests to measure how well your lungs work

If the specific ILD diagnosis remains uncertain despite these tests, our pulmonologists may recommend a biopsy. For this test, we remove a small sample of your lung tissue and examine it under a microscope to determine what type of ILD you may have.