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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.

What is Interstitial Lung Disease?

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 active and informed steps in your 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 diseases. Understanding which one you have helps your healthcare provider plan the best care for you.

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What Are the Different Types of ILD?

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

  • Asbestosis — caused by exposure to high levels of asbestos fibers
  • Connective Tissue Disease-Associated ILD (CTD-ILD) – develops in people with autoimmune diseases such as scleroderma and rheumatoid arthritis.
  • Coal Workers Pneumoconiosis — develops when coal dust is inhaled over a long period of time (also called "black lung disease")
  • Cryptogenic Organizing Pneumonia — a rare type of ILD with no specific known cause
  • Hypersensitivity Pneumonitis — caused by inhaling environmental allergens such as mold, animal dander and bird droppings
  • Idiopathic Pulmonary Fibrosis (IPF) — a progressive form of pulmonary fibrosis with unknown cause and one of the most common types of ILD
  • Pulmonary Fibrosis (PF) — a general term for  interstitial lung disease that have developed scarring in the lungs, IPF is the most common form of PF
  • Sarcoidosis — an inflammatory disease that can affect multiple organs, including the lungs
  • Silicosis — caused by inhaling silica dust, a mineral found in rocks, sand and soil

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

What Causes ILD?

There are dozens of known causes of interstitial lung disease, but in some cases, a specific cause cannot be identified. Some causes and risk factors include:

  • 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 (such as exposure to bird feathers or droppings or mold)
  • Autoimmune (your immune system attacks the body)
  • Occupational exposure (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

ILD symptoms may be present right away in some cases, but are usually mild at first and then may get worse over months or years.

  • Clubbing or widening of the fingers or toes
  • Weight loss
  • Loss of appetite
  • Low oxygen levels
  • Lung sounds called “crackles”

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. It is important to share information about any other medical conditions you have and your work and hobbies that may result in toxic exposure. 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 specific type of ILD, its severity and how the disease is affecting your daily life. Treatment goals include reducing symptoms, improving quality of life, maintaining lung function and slowing disease progression. 

Anti-inflammatory medications like corticosteroids are used to reduce inflammation in the lungs. This type of medication is often prescribed if you have certain inflammatory or autoimmune-related forms of ILD. 

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

If you have progressive pulmonary fibrosis (PPF), your healthcare provider may recommend medicines such as nintedanib or nerandomilast to help slow lung scarring and protect your lung function. It is important to see your pulmonologist regularly, and seek out one who has experience treating ILD. Regular visits help your healthcare provider track how your condition is changing, make sure your treatment is working and talk with you about new treatment options or clinical trials that may be a good fit for you.

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.

A 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. Sometimes, only one lung is replaced with a healthy donor lung, and the other lung remains in place. A successful transplant 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. Vaccines can help protect against respiratory infections that may worsen ILD symptoms and affect your overall lung health.

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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