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A Legacy of awareness

A diagnosis of ILD brings with it a host of questions for patients and their families. Erin Popovich wanted to make it easier for people with ILD to get access to specialists and second opinions. Erin’s legacy lives on through the Erin Popovich Endowment.

The progress and treatment of ILD will be different depending on the type you have. That is why your doctor will perform a thorough evaluation and likely order tests to search for a cause.

Healthy lung

Lung icon
Healthy Lung icon

ILD lung

Unhealthy lung image 3

Unhealthy lung image 2
Unhealthy lung image 1

Types of ILD

There are many types of ILD. Those with similar causes or findings are grouped together.

  • Pneumoconioses

    Pneumoconioses is any lung disease, including ILD, caused by exposure to certain inorganic or organic dust particles. These harmful substances can be in places where you spend a lot of time, such as your work, but they can also be environmental.

    Pneumoconioses include:

    Some harmful substances that may cause ILD are:

    • Beryllium
    • Birds or bird feathers (in down blankets, coats, and pillows)
    • Dusts, such as aluminum, antimony, barium, graphite, iron, kaolin, mica, or talc
  • Workplace Exposures by Job

    ASBESTOSConstruction worker (insulation); shipyard worker; mechanic (brake pads)
    SILICASand blaster
    COAL DUSTCoal miner
    BERYLLIUMDenture manufacturer
    GRAIN DUSTPeanut industry worker
    cobalt, tungsten, etc.
  • Granulomatous

    Granulomas are collections of cells that form in some inflammatory conditions. They can occur all over the body. When granulomas form in the lung tissue, different signs and symptoms of ILD can appear.

    Some granulomatous conditions that can lead to ILD are:

  • Collagen Vascular Disease

    Autoimmune disease and connective tissue disease cause the immune system to attack the body. If an autoimmune disease targets the lung, it can cause inflammation and scarring of lung tissue. Sometimes people don’t even know they have an autoimmune disorder until after they develop ILD.

    Autoimmune diseases that can cause ILD include:

  • Drug Toxicity (Medication)

    Some medications play a role in the development of ILD. Factors include the:

    • Type of medicine
    • Amount of time you take it
    • Dosage 

    Drugs that can lead to ILD include:

    • Antibiotics (nitrofurantoin)
    • Cancer treatments (bleomycin) and chemotherapy
    • Heart medications (amiodarone)
    • Immunotherapy drugs (nivolumab, pembrolizumab)
    • Medications for rheumatoid arthritis
    • Methotrexate
    • Nitrofurantoin
  • Radiation Treatment

    Radiation helps treat many cancers. But it sometimes causes ILD. People can develop ILD weeks or years after having radiation treatments.

  • Idiopathic ILD (No Known Cause)

    More than half of ILD cases are idiopathic. This means they have no known cause. IPF is the best example of an idiopathic ILD. Idiopathic ILDs can be associated with smoking or other rare causes. Sometimes they are unclassifiable.

    The types of idiopathic ILD are:

    Acute idiopathic ILDs usually have symptoms like pneumonia, but they are not infections. Doctors do not treat acute ILDs with antibiotics.

  • Chronic Fibrosing

    Chronic fibrosing ILDs may take time to cause symptoms. By the time someone has symptoms, the lung already has scarring that is not reversible. Idiopathic pulmonary fibrosis (IPF) is one type of a chronic fibrosing ILD with no identifiable cause (some known causes can also trigger fibrosis). Another type is chronic nonspecific interstitial pneumonia (NSIP).

  • Rare Idiopathic ILD

    Some ILDs are extremely rare, but they have specific features that distinguish them from other ILDs. Rare idiopathic ILDs include:

    • Lymphoid interstitial pneumonia (LIP)
    • Pleuroparenchymal fibroelastosis (PPFE)
  • Unclassifiable ILD

    Some ILDs do not fall into any particular group. They can still be treated. Your doctor may recommend a lung biopsy to help determine your treatment options.

  • Smoking-Related ILD

    Smoking rarely causes ILD. If a smoker develops ILD, the first treatment step is to stop smoking. ILDs caused by smoking are:

  • Familial ILD (Genetic)

    Some forms of ILD tend to run in families. For example, idiopathic pulmonary fibrosis (IPF) runs in families 10% to 15% of the time.

    Genetic changes that cause IPF are passed down from a parent to a child. Genetic tests for IPF are not a part of a routine workup. Ask your lung doctor about testing if more than one person in your family has pulmonary fibrosis.

Diagnosing ILD

Diagnosing ILD image

Diagnosing ILD

The causes of ILD can be hard to find and vary depending on the type of ILD. Many times, doctors cannot identify the cause of ILD. In those cases, we say ILD is idiopathic.

Sometimes ILD is caused by another disease, like rheumatoid arthritis. Other times, it is caused by something:

Find out more about diagnosing ILD and ways you can be prepared for your appointment.

“Living with Interstitial Lung Disease” patient education guide

This 52-page guide explores every facet of ILD that you may encounter, from diagnosis and treatment to support and myths. With the most up-to-date information available, this guide will help you and your loved ones feel confident when making decisions about your diagnosis.

ILD Patient Guide