Interstitial Lung Disease (ILD): Everything You Need to Know

Rachael Zimlich, BSN, RN
·8 min read

Medically reviewed by Sanja Jelic, MD

Interstitial lung disease (ILD) is a group of more than 200 lung disorders that can cause damage and scarring to these organs. Over time, the effects of these diseases can limit your breathing and lead to respiratory failure and even death.

This article will review some of the more common forms of interstitial lung disease, their causes and risk factors, available treatments, and how ILD can impact your overall well-being and life span.

<p>Edwin Tan / Getty Images</p>

Edwin Tan / Getty Images

Types of Interstitial Lung Disease

Many lung diseases cause damage and scarring in the lungs. Each of these conditions has unique symptoms and mechanisms, but they are grouped under the umbrella of ILD for the way the damage to lung tissues affects your breathing.

With most forms of ILD, much of the damage occurs as scarred and destroyed alveoli (air sacs) or stiffening and thickening of lung tissue.

Although ILD can take many forms, most of these conditions are rare. The most commonly diagnosed forms of ILD are:

  • Idiopathic pulmonary fibrosis: This chronic form of ILD develops when your lung tissue becomes thick and stiff for an unknown reason. Smoking and age may contribute to this problem, which can make it more difficult to breathe as scarring develops and lung tissue is damaged.

  • Hypersensitivity pneumonitis: This type of ILD is a rare immune reaction that appears in response to environmental or chemical irritants. This irritation leads to short- or long-term inflammation, making breathing difficult.

  • Connective tissue disease: Some connective tissue disorders can affect your lungs. While lung damage isn't the primary symptom of many of these conditions, damage can still impact your breathing. Some connective tissue disorders affecting the lungs include rheumatoid arthritis, systemic sclerosis, and idiopathic inflammatory myopathy.

  • Sarcoidosis: Sarcoidosis is a condition in which growths called granulomas form throughout the body in response to inflammation. While sarcoidosis and ILD are two different conditions, these conditions commonly co-occur. More research is needed to determine the link between these two conditions.

Related: Types of Lung Disease

Interstitial Lung Disease Symptoms

There are distinct differences between the various forms of ILD, but there are also common threads. Some symptoms that often appear across the ILD spectrum are:

Related: Hypoxemia (Low Blood Oxygen)

Interstitial Lung Disease Causes

Just as there are multiple types of ILD, there are various causes and risk factors. These include genetic mutations, environmental exposures, certain medications, and other systemic health conditions.

Some forms of ILD do not have a clear cause and usually include the term "idiopathic" (unknown cause) in the name. For example, idiopathic pulmonary fibrosis is lung inflammation and scarring without a known cause.

The section that follows includes categories of causes and the most common types of ILD associated with each cause.

Autoimmune Conditions

When you have an autoimmune disease, your body starts to attack its own tissues. There are many forms of autoimmune disease, including types that affect the lungs.

Autoimmune diseases that are often linked to or associated with interstitial lung disease include:

You may be diagnosed with a form of ILD specific to these conditions, or your lung damage could be considered a part of the overall disease.

Exposure to Toxic Substances or Irritants

Toxic environmental exposures are also linked to lung diseases. Irritating chemicals, air pollution, and extreme climate changes can contribute to breathing problems.

Many kinds of chemicals and toxins can damage lung tissue, but the types of ILD most often linked to exposures include:

Some occupations and lifestyle choices can also lead to toxin-related ILD. This includes working with paints, asbestos, construction materials, or smoking cigarettes.

Medications

Some medications that are highly valuable at treating specific conditions may also cause problems in other parts of the body. Chemotherapy and radiation are prime examples.

Treatments that target certain problems but have a high likelihood of also damaging healthy tissues and causing ILD include:

  • Chemotherapy

  • Radiation

  • Certain antibiotics

  • Some heart medications

Interstitial Lung Disease Diagnosis

A diagnosis of ILD is usually made by a multidisciplinary team of healthcare providers that can include the following professionals, among others:

  • Primary care provider

  • Pulmonologist (lung specialist)

  • Rheumatologist (specialist in inflammatory and musculoskeletal diseases)

Most cases of ILD are idiopathic, but diagnostic techniques are standard regardless of the type of ILD you have.

A healthcare provider will begin the diagnostic process by reviewing your personal and family medical histories, current and past medications, and symptoms. Then, they will assess your overall health and body functions.

In some cases, you may require additional testing to confirm a diagnosis of ILD or identify a specific cause. Testing can include the following:

Read Next: What Is an FEV1 Test?

Treatment Options for Interstitial Lung Disease

Specific treatment strategies will depend on your health and symptoms.

If your ILD was diagnosed early, you may be able to stop or slow the progression of your lung damage through regular lung capacity monitoring.

In more advanced cases, treatment will focus on alleviating symptoms and complications of ILD, such as shortness of breath and low blood oxygen levels. Beyond that, treatments will vary depending on your type of ILD and the underlying cause.

For treating shortness of breath and other symptoms of ILD, a healthcare provider may recommend one or more of the following treatments:

You may also need to examine your environment and lifestyle and consider things like quitting smoking or changing your work environment.

If your ILD is known to have been caused by another systemic condition or autoimmune disease, a healthcare provider will recommend additional treatments to control the underlying problem.

In the most severe cases of ILD that progress to respiratory failure, you may require aggressive oxygen therapy and breathing support or even consider lung transplantation.

Interstitial Lung Disease Risk Factors

Not every case of ILD has a clear cause, but there are risk factors that could increase your chances of developing lung damage.

Some risk factors for ILD are:

  • Smoking

  • Prior lung infections, including from COVID-19

  • Family history of lung disease

  • Occupational or residential exposures to dust, mold, and other toxins

  • Autoimmune conditions

  • Connective tissue disorders

Living With Interstitial Lung Disease

ILD symptoms can vary by type and severity, but many people with conditions that fall into this category end up with symptoms that significantly impact their daily life.

Regular, ongoing monitoring and care can also help you maximize your quality of life with ILD. Ongoing care includes:

  • Attending regular appointments with your healthcare team

  • Avoiding toxins and other triggers

  • Staying up-to-date on vaccines, especially those that prevent respiratory viruses

  • Controlling conditions—such as heart disease—that can put stress on your lungs

Even with the best treatment strategies, ILD is associated with a decreased quality of life and can limit your abilities in many ways. Your healthcare team can provide a list of local resources and support groups that may help.

It's important to understand the full nature of your disease and what a worsening condition might look like. Sometimes, this may involve working with providers of palliative care (specialized care for people living with serious illness) or hospice care (end-of-life care).

Interstitial Lung Disease Complications

Some complications that can develop from various forms of ILD include:

Interstitial Lung Disease Life Expectancy and Outlook

Even with treatment, ILD can be a severe and often fatal condition. One study estimates that 40% of people diagnosed with ILD die from the condition or its complications within 10 years of diagnosis.

Survival and general outlook can vary by the type of ILD you have, but generally, every form of the disease is progressive and fatal in its later stages. Even with treatment, ILD can lead to death in as little as three to five years.

Those who survive longer may have been able to receive a lung transplant. When ILD does become fatal, flare-ups and pneumonia are the most common causes.

How to Prevent Interstitial Lung Disease

You can't prevent every type of ILD, especially those linked to family history, genetics, and autoimmune diseases. Healthy lifestyle choices can help you avoid preventable causes, though.

Steps to lower your risk of developing ILD include:

  • Quitting smoking

  • Avoiding secondhand smoke

  • Living in an area with low pollution

  • Avoiding careers in which irritants are common (e.g., construction, painting, etc.)

  • Getting regular checkups

  • Staying current on vaccinations

Summary

Interstitial lung disease is an umbrella term for the many conditions that cause scarring in your lungs. When your lungs are injured and scarred, they become less effective in moving oxygen and other gases in and out of your body. Even with treatment, many forms of ILD are fatal within a few years of diagnosis.

If you know you have a family history of ILD, talk to your healthcare provider about steps you can take to prevent the condition from flaring up or worsening. If you've already been diagnosed with ILD, your healthcare provider can help you manage your symptoms, including when they worsen.

Read the original article on Verywell Health.