Interstitial Lung Disease (ILD)

A group of lung conditions that cause inflammation or scarring, making it harder to breathe.

Young couple hold hands and look at each other in doctor's office with older doctor looking on. Young couple hold hands and look at each other in doctor's office with older doctor looking on.

Interstitial lung disease (ILD) is not a single disease—it's a term used for many conditions that affect the tissue in and around the air sacs of the lungs. Over time, this damage can make breathing more difficult and reduce how much oxygen gets into your body.

ILD is different for everyone, and learning more about your specific condition can help you take the next steps in care.

Key Facts about ILD

  • An ILD is a type of restrictive lung disease, meaning the damage in your lungs makes it harder for your lungs to fully expand or fill up with air. 
  • Shortness of breath is the most common symptom.
  • Anyone can get ILD, including children. 
  • Idiopathic pulmonary fibrosis, IPF, is the most common type.
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There are different kinds of chronic lung disease. Understanding which one you have helps you and your healthcare provider plan the best care for you.

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Types of ILD

Interstitial lung disease includes many different conditions. Some of these include:

Visit the Lung Disease Lookup to learn more about symptoms, causes, diagnosis, treatments and management for chronic lung conditions.

What Causes ILD?

There are dozens of known causes, but an ILD can also happen without a cause.

  • Medication induced (certain medications such as chemotherapy or other medications that affect the lungs) 
  • Genetic (passed down from another family member)
  • Radiation induced (past or present radiation to your chest)
  • Environmental (exposure to animal proteins or mold)
  • Autoimmune (your immune system attacks the body)
  • Occupational (past or present exposure to dust, chemicals, fumes, and/or vapors)
  • Idiopathic (a cause cannot be found)

Lung infections and exposure to tobacco smoke may cause or worsen an ILD. 

Symptoms of ILD

Common symptoms:

Advanced symptoms:

  • Clubbing or widening of the fingers or toes
  • Weight loss
  • Loss of appetite
  • Low oxygen levels
  • Lung sounds called “crackles”
Severe cases that are left untreated can develop life-threatening complications including high blood pressure, heart or respiratory failure.

How Is ILD Diagnosed

Diagnosing ILD often involves multiple steps and tests.

To diagnose an ILD, your healthcare provider will ask you about your family and medical history and do a physical exam. A chest X-ray or CT scan can get a better look at your lungs and help diagnose ILD. Other tests may include a blood test to check for autoimmune diseases, an arterial blood gas test or pulse oximetry can measure your blood oxygen levels, pulmonary exercise tests and lung function tests to see how well your lungs work. 

To diagnose a specific type of ILD, your healthcare provider may order more invasive procedures such as a bronchoscopy or a lung biopsy

Treating ILD

Treatment for ILDs varies depending on the type of ILD diagnosed and the severity. Treatment goals include reducing symptoms, improving quality of life and slowing the disease's progression. 

Anti-inflammatory medications like corticosteroids are used to reduce inflammation in the lungs. This type of medication is prescribed if you have an autoimmune disease. 

For people diagnosed with idiopathic pulmonary fibrosis, IPF, there are two FDA medications to help slow the progression of new scarring: pirfenidone and nintedanib.

Oxygen is needed to keep every organ in your body healthy. When you are not getting enough oxygen, your healthcare provider will prescribe supplemental oxygen.

lung transplant may be a treatment option if you have a severe and progressive form of ILD, especially IPF. To qualify for a lung transplant, you will need to go through an extensive evaluation process and if approved you will be put on a wait list. 

During the lung transplant, either one or both diseased lungs are replaced with healthy lungs from a donor. Double lung transplants are more common. If you receive one lung from a donor, your other lung will remain in your chest. If the surgery is successful, it can improve your quality of life and life expectancy.


Managing ILD

You can take steps to manage your lung health and improve your symptoms. 

Talk to your healthcare provider about enrolling in a pulmonary rehabilitation program. This program can help you meet new people, exercise and learn how to improve your lung health. Pulmonary rehab covers topics like breathing exercises, nutrition, and improving your mental and emotional health. 

If you smoke, use other tobacco products or vape, quit as soon as possible and avoid secondhand smoke. The American Lung Association offers programs to help you quit

Ask your healthcare provider about recommended vaccinations

Physical activity and exercise can boost your energy levels, improve how your body uses oxygen, and help to reduce stress, anxiety and depression. Talk to your healthcare provider about getting started with an exercise routine.

Diet and a healthy weight play an important role in managing an ILD. Some medications to treat ILDs cause side effects like diarrhea or acid reflux. Ask your healthcare provider or a nutritionist about ways to manage any challenges like bloating, acid reflux or getting short of breath. 

Webinars

In this patient‑focused webinar, pulmonologist Erica Farrand, MD (UCSF) and patient Laura R. discuss living with idiopathic pulmonary fibrosis, including symptoms, diagnosis, and management.

Managing an interstitial lung disease (ILD) can feel overwhelming. In this patient‑focused webinar, hear from pulmonologist Erica Farrand, MD (UCSF), and Demi, who is living with ILD.

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