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What is Interstitial Lung Disease?

Interstitial lung disease (ILD) is a group of rare lung conditions that can cause inflammation or scarring in the lungs, making it harder to breathe.

ILD can have different causes, including exposure to mold or harmful substances at work or in your home, genetics, or even autoimmune diseases like rheumatoid arthritis or scleroderma. No matter the cause, ILD can affect your lung function and, in some cases, lead to lasting damage.

While ILD isn’t as common as other lung diseases, it’s important to catch it early to help manage the symptoms and protect your lungs.

Types of Interstitial Lung Disease

There are many different types of ILDs, each with its own characteristics. Here are some of the more common ones:

• Idiopathic pulmonary fibrosis (IPF), the most common type of ILD, involves progressive scarring of the lungs, with no known underlying cause. Over time, this scarring can make it harder for the lungs to provide the body with enough oxygen. While the exact cause is unknown, IPF has been linked to certain environmental exposures, such as pollutants or dust, and people with a history of smoking. IPF is most commonly diagnosed in people over age 50.
• Systemic autoimmune rheumatic diseases (SARDs) are chronic conditions that occur when the immune system attacks healthy tissues throughout the body. These diseases can affect multiple organs, including the joints, skin, muscles, heart and kidneys. Common SARDs include rheumatoid arthritis, scleroderma and Sjögren's syndrome.
• Connective tissue disease-associated ILD develops when an autoimmune condition, like SARD, causes inflammation and scarring in the lungs. This can make breathing more difficult over time. Conditions such as scleroderma, rheumatoid arthritis, lupus, Sjögren's syndrome and myositis can lead to this form of ILD.
• Sarcoidosis involves small areas of inflammation that can occur throughout the body’s organs, but most commonly in the lung. It can also affect your eyes, heart, liver, kidneys, skin, lymph nodes and brain.
• Hypersensitivity pneumonitis (HP) is related to environmental exposure. It can be due to your lungs’ reaction to inhaled particles, or antigens, like mold and airborne dust.
• Nonspecific interstitial pneumonia can be caused by an autoimmune disease, drug reactions or environmental exposure. In some cases, no cause is found.

How We Diagnose Interstitial Lung Disease

At Rush, diagnosing ILD starts with a thorough conversation with a pulmonologist about your symptoms, medical history and daily environment. They will ask you when your symptoms started and whether you have experienced issues like shortness of breath, fatigue or joint pain, or rashes and dry eyes, which may indicate an autoimmune condition.

Your pulmonologist will also ask you questions about your work and home environment, like whether you have worked around dust, chemicals or fumes, or if you have been exposed to mold. Understanding these factors helps us determine whether external exposures could be contributing to lung inflammation or scarring.

Next, you’ll undergo blood tests, to check for autoimmune conditions, and a high-resolution CT scan, which our expert thoracic radiologists will review for a detailed look at your lungs.

If a diagnosis isn’t clear at this point, we will bring together a team of specialists to review the findings from your tests and our evaluations. This collaborative approach allows us to make the most informed decision about your diagnosis and care plan.

In rare cases, we may recommend a lung biopsy. This involves taking a small piece of lung tissue for closer examination under a microscope to confirm your diagnosis.

The Causes of Interstitial Lung Disease

ILD can develop for a variety of reasons, but in many cases, the exact cause is unknown.

Here are some of the most common factors that can cause ILD:

• Smoking. Smoking is a major risk factor for developing ILD, specifically for idiopathic pulmonary fibrosis (IPF). While the exact cause of IPF is often unknown, smoking can contribute to lung damage and increase the risk of developing scarring and inflammation in the lungs.
• Autoimmune diseases. Some forms of ILD are linked to autoimmune conditions that cause inflammation in the body, including:
  • Rheumatoid arthritis
  • Scleroderma
  • Lupus
  • Sjögren’s syndrome
  • Other conditions that cause joint pain, muscle pain or rashes
• Environmental and occupational exposures. Breathing in harmful substances over time can lead to lung inflammation and scarring. These exposures can come from:
  • Workplace settings (dust, vapors, fumes, chemicals)
  • Mold exposure in the home
• Genetics and family history. Some people develop familial pulmonary fibrosis, which tends to appear about 10 years earlier than nonfamilial cases. If multiple family members have had an ILD, genetics may play a role.
• Age and risk factors. ILD is most often diagnosed in people in their 50s and 60s. Autoimmune-related ILD can appear earlier, sometimes in people in their 40s, while sarcoidosis, another form of ILD, tends to develop in people in their 20s or 30s.

How We Treat Interstitial Lung Disease

While ILD-related lung damage can’t be reversed, our team of experts focuses on the least invasive treatment options to help you manage your symptoms, prevent further progression and improve quality of life.

Your pulmonologist may recommend one or more of the following treatment options:

• Medications:
  • Antifibrotic medications can help slow or stop lung scarring, though they don’t reverse existing damage or improve symptoms.
  • Anti-inflammatory medications can be used to manage autoimmune-related inflammation in ILD.
  • Immunosuppressants can be used to prevent the immune system from attacking the lungs for ILDs caused by an autoimmune disease.
  • Inhaled medications may help reduce strain on the heart and potentially slow lung scarring, as some ILD-related lung damage can stress the heart and lead to pulmonary hypertension.
• Respiratory support:
  • Supplemental oxygen may be needed to help you breathe if your lung function has declined significantly.
• Pulmonary rehabilitation:
  • Pulmonary rehab is a structured exercise program that can help improve your breathing and overall fitness. We have pulmonary rehab programs at Rush Oak Park Hospital and Rush Copley Medical Center.

Second Opinion ÍÑÒÂÖ±²¥ for Interstitial Lung Disease

If you’re coming to Rush for a second opinion, our team will carefully review your previous test results and any medical records to ensure you’re getting the most accurate diagnosis and best treatment plan.

To help you prepare for your appointment, you will want to gather and plan to bring with you the following items:

• CT scan images: If you’ve had a CT scan of your lungs at another hospital, please bring the images on a disk. This provides a clearer image of your condition for our radiologists to review.
• Pulmonary function test (PFT) results: If you have previous PFT results, please bring copies to help us understand and track how your lung is functioning.
• Lab results: Your lab results can provide additional insights into your condition.
• Medication history: Gather any information about the medications you’ve tried in the past to treat your ILD, including what has worked and what hasn't.

If we need more information to confirm your diagnosis, we may recommend additional testing to give us a complete understanding of your condition.