Interstitial lung disease

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What is interstitial lung disease?

Interstitial lung disease (ILD) is a term that groups together more than 200 types of lung disorders. All ILD diagnoses involve inflammation and scarring (fibrosis) in the lungs.

ILD can cause shortness of breath, cough, fatigue, weight loss and pain. It can be related to allergic exposure, environmental exposure, autoimmune disease or other causes. It can also have no known cause, which is called idiopathic pulmonary fibrosis (IPF). Without treatment, ILD worsens over time and can cause life-threatening complications. ILD may also occur in people with a connective tissue disease. This form of ILD is called connective tissue disease-associated ILD (CTD-ILD).

If you were diagnosed with lung disease, it's important to see an experienced specialist. An accurate ILD diagnosis leads to an individualized treatment plan that helps you breathe easier.

ILD symptoms

Unlike conditions like asthma or bronchitis, which primarily affect the airways, ILD directly impacts the lung tissue itself.

Common symptoms include:

  • Shortness of breath: This is often the first and most noticeable symptom, especially with physical activity. 
  • Dry cough: A persistent cough that doesn't produce phlegm.
  • Fatigue: Feeling tired and weak, even with adequate rest.
  • Chest pain: A sharp or aching pain in the chest.
  • Rapid breathing: Breathing quickly, even at rest.

Causes of interstitial lung disease

The exact cause of ILD can vary, but some common factors include:

  • Exposure to environmental toxins: Substances like asbestos, silica, and certain chemicals can damage lung tissue.
  • Autoimmune diseases: Conditions like rheumatoid arthritis, lupus, and scleroderma can lead to inflammation and scarring of the lungs.
  • Medications: Certain drugs can cause lung damage as a side effect.
  • Genetics: Some forms of ILD, such as idiopathic pulmonary fibrosis, have a genetic component.
  • Infections: Viral or fungal infections can sometimes lead to ILD.

How is interstitial lung disease diagnosed?

During your initial visit, your team will ask questions about your medical history, exposures, family history and symptoms. Then, your ILD specialist may order some tests.

Treatments for interstitial lung disease

Treatment can often-times help improve or slow down the progression of ILD. The choice of treatment may not be straight forward. We will discuss your options with you and what you can expect from potential treatments.

The main goals of treatment are to:

  • Slow disease progression and improve lung function when possible
  • Treat underlying causes of lung damage
  • Reduce inflammation in the lungs and slow down scarring
  • Minimize symptoms so you can do more of the things you love

Your treatment will depend on the type of ILD you have and how severe your symptoms are. Your care team will use information from your ILD diagnosis, including imaging and biopsy results, to make a treatment plan that's right for you.

Expert care for ILD at Penn Medicine

ILD varies from person to person, so treatment needs to be as unique as you are. Our patients receive accurate and detailed diagnoses, leading to the most effective treatment plans. Your care team then develops a personalized treatment plan based on the exact type of ILD you have and how severe it is. When you come to the Penn ILD and Sarcoidosis program, you'll find:

  • Experienced, specialized team: At Penn, you don't just see a doctor — you see a whole team. Your diagnostic team includes pulmonologists, chest radiologists, lung pathologists and nurses who specialize in interstitial lung disease. They collaborate with medical geneticists and surgeons who specialize in these conditions. Our experts often review your case together to make sure you get the right diagnosis.
  • Focus on noninvasive diagnosis: We see patients with ILD every day, so we recognize the patterns. We use the most advanced technology to get detailed images of your lungs. These images are then reviewed by expert radiologists. We also perform specific blood tests to diagnose certain rare lung diseases. This means we can often make an accurate diagnosis without invasive surgical biopsy.
  • Access to new ILD treatments and innovative research: As one of the world's leading ILD programs, we can offer patients the latest treatments aimed at slowing the progression of the disease. We work on your behalf to get you access to and approval for these therapies, so you can focus on healing. Our team conducts ongoing research into the causes of ILD and the best ways to treat it. Our involvement in clinical trials means we're able to offer patients access to leading-edge treatments still in development.
  • Supportive services: At Penn, you're surrounded by support. Support groups and smoking cessation programs help you live better with ILD. Our social workers help you access any services you might need. Palliative care experts help you manage symptoms of ILD and feel your best.

Related specialties

Pulmonary and respiratory care
Explore expert pulmonology care with advanced treatments and a team of specialized clinicians.
ILD and Sarcoidosis Program
Receive expert diagnosis and personalized care for interstitial lung disease and sarcoidosis.

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