Table of ContentsView AllTable of ContentsTypesSymptomsCausesDiagnosisTreatmentRisk FactorsLiving With ILDComplciationsOutlookPrevention
Table of ContentsView All
View All
Table of Contents
Types
Symptoms
Causes
Diagnosis
Treatment
Risk Factors
Living With ILD
Complciations
Outlook
Prevention
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 torespiratory failureand 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.
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 destroyedalveoli(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:
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:
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 fibrosisis 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.
Lung Scarring: What to Know
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:
Interstitial Lung Disease Diagnosis
Most cases of ILD are idiopathic, but diagnostic techniques are standard regardless of the type of ILD you have.
In some cases, you may require additional testing to confirm a diagnosis of ILD or identify a specific cause. Testing can include the following:
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.
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:
Living With Interstitial Lung Disease
Regular, ongoing monitoring and care can also help you maximize your quality of life with ILD. Ongoing care includes:
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.
Those who survive longer may have been able to receive alung 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:
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.
18 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Allergy and Asthma Network.Interstitial lung diseases.National Institutes of Health.What are interstitial lung diseases?.National Institutes of Health.Idiopathic pulmonary fibrosis.National Institutes of Health.Hypersensitivity pneumonitis.American Thoracic Society.Connective-tissue related interstitial lung disease primer.Bergantini L, Nardelli G, d’Alessandro M, et al.Combined sarcoidosis and idiopathic pulmonary fibrosis (CSIPF): A new phenotype or a fortuitous overlap? Scoping review and case series.J Clin Med.April 2022;11(7):2065. doi:10.3390/jcm11072065National Institutes of Health.Sarcoidosis.National Institutes of Health.Interstitial lung diseases: symptoms.University of California San Francisco.What are different types of ILD?.National Institutes of Health.Interstitial lung diseases: causes and risk factors.Antoine MH, Mlika M.Interstitial lung disease.StatPearls.2023.National Institutes of Health.Interstitial lung diseases: treatment.Wijsenbeek M, Suzuki A, Maher TM.Interstitial lung diseases.Lancet. 2022;400(10354):769-786. doi:10.1016/S0140-6736(22)01052-2National Institutes of Health.Interstitial lung disease: living with.Kahlmann V, Moor CC, Wijsenbeek MS.Managing fatigue in patients with interstitial lung disease.Chest. November 2020;158(5):2026-2033. doi:10.1016/j.chest.2020.04.047Galioto F, Palmucci S, Astuti GM, et al.Complications in idiopathic pulmonary fibrosis: focus on their clinical and radiological features.Diagnostics. 2020;10(7):450. doi:10.3390/diagnostics10070450Perelas A, Silver RM, Arrosi AV, Highland KB.Systemic sclerosis-associated interstitial lung disease.Lancet. 2020;8(3):304-320. doi:10.1016/S2213-2600(19)30480-1Chen X, Guo J, Yu D, Jie B, Zhou Y.Predictors of mortality in progressive fibrosing interstitial lung diseases.Front Pharmacol. October 2021;12:754851. doi:10.3389/fphar.2021.754851
18 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Allergy and Asthma Network.Interstitial lung diseases.National Institutes of Health.What are interstitial lung diseases?.National Institutes of Health.Idiopathic pulmonary fibrosis.National Institutes of Health.Hypersensitivity pneumonitis.American Thoracic Society.Connective-tissue related interstitial lung disease primer.Bergantini L, Nardelli G, d’Alessandro M, et al.Combined sarcoidosis and idiopathic pulmonary fibrosis (CSIPF): A new phenotype or a fortuitous overlap? Scoping review and case series.J Clin Med.April 2022;11(7):2065. doi:10.3390/jcm11072065National Institutes of Health.Sarcoidosis.National Institutes of Health.Interstitial lung diseases: symptoms.University of California San Francisco.What are different types of ILD?.National Institutes of Health.Interstitial lung diseases: causes and risk factors.Antoine MH, Mlika M.Interstitial lung disease.StatPearls.2023.National Institutes of Health.Interstitial lung diseases: treatment.Wijsenbeek M, Suzuki A, Maher TM.Interstitial lung diseases.Lancet. 2022;400(10354):769-786. doi:10.1016/S0140-6736(22)01052-2National Institutes of Health.Interstitial lung disease: living with.Kahlmann V, Moor CC, Wijsenbeek MS.Managing fatigue in patients with interstitial lung disease.Chest. November 2020;158(5):2026-2033. doi:10.1016/j.chest.2020.04.047Galioto F, Palmucci S, Astuti GM, et al.Complications in idiopathic pulmonary fibrosis: focus on their clinical and radiological features.Diagnostics. 2020;10(7):450. doi:10.3390/diagnostics10070450Perelas A, Silver RM, Arrosi AV, Highland KB.Systemic sclerosis-associated interstitial lung disease.Lancet. 2020;8(3):304-320. doi:10.1016/S2213-2600(19)30480-1Chen X, Guo J, Yu D, Jie B, Zhou Y.Predictors of mortality in progressive fibrosing interstitial lung diseases.Front Pharmacol. October 2021;12:754851. doi:10.3389/fphar.2021.754851
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Allergy and Asthma Network.Interstitial lung diseases.National Institutes of Health.What are interstitial lung diseases?.National Institutes of Health.Idiopathic pulmonary fibrosis.National Institutes of Health.Hypersensitivity pneumonitis.American Thoracic Society.Connective-tissue related interstitial lung disease primer.Bergantini L, Nardelli G, d’Alessandro M, et al.Combined sarcoidosis and idiopathic pulmonary fibrosis (CSIPF): A new phenotype or a fortuitous overlap? Scoping review and case series.J Clin Med.April 2022;11(7):2065. doi:10.3390/jcm11072065National Institutes of Health.Sarcoidosis.National Institutes of Health.Interstitial lung diseases: symptoms.University of California San Francisco.What are different types of ILD?.National Institutes of Health.Interstitial lung diseases: causes and risk factors.Antoine MH, Mlika M.Interstitial lung disease.StatPearls.2023.National Institutes of Health.Interstitial lung diseases: treatment.Wijsenbeek M, Suzuki A, Maher TM.Interstitial lung diseases.Lancet. 2022;400(10354):769-786. doi:10.1016/S0140-6736(22)01052-2National Institutes of Health.Interstitial lung disease: living with.Kahlmann V, Moor CC, Wijsenbeek MS.Managing fatigue in patients with interstitial lung disease.Chest. November 2020;158(5):2026-2033. doi:10.1016/j.chest.2020.04.047Galioto F, Palmucci S, Astuti GM, et al.Complications in idiopathic pulmonary fibrosis: focus on their clinical and radiological features.Diagnostics. 2020;10(7):450. doi:10.3390/diagnostics10070450Perelas A, Silver RM, Arrosi AV, Highland KB.Systemic sclerosis-associated interstitial lung disease.Lancet. 2020;8(3):304-320. doi:10.1016/S2213-2600(19)30480-1Chen X, Guo J, Yu D, Jie B, Zhou Y.Predictors of mortality in progressive fibrosing interstitial lung diseases.Front Pharmacol. October 2021;12:754851. doi:10.3389/fphar.2021.754851
Allergy and Asthma Network.Interstitial lung diseases.
National Institutes of Health.What are interstitial lung diseases?.
National Institutes of Health.Idiopathic pulmonary fibrosis.
National Institutes of Health.Hypersensitivity pneumonitis.
American Thoracic Society.Connective-tissue related interstitial lung disease primer.
Bergantini L, Nardelli G, d’Alessandro M, et al.Combined sarcoidosis and idiopathic pulmonary fibrosis (CSIPF): A new phenotype or a fortuitous overlap? Scoping review and case series.J Clin Med.April 2022;11(7):2065. doi:10.3390/jcm11072065
National Institutes of Health.Sarcoidosis.
National Institutes of Health.Interstitial lung diseases: symptoms.
University of California San Francisco.What are different types of ILD?.
National Institutes of Health.Interstitial lung diseases: causes and risk factors.
Antoine MH, Mlika M.Interstitial lung disease.StatPearls.2023.
National Institutes of Health.Interstitial lung diseases: treatment.
Wijsenbeek M, Suzuki A, Maher TM.Interstitial lung diseases.Lancet. 2022;400(10354):769-786. doi:10.1016/S0140-6736(22)01052-2
National Institutes of Health.Interstitial lung disease: living with.
Kahlmann V, Moor CC, Wijsenbeek MS.Managing fatigue in patients with interstitial lung disease.Chest. November 2020;158(5):2026-2033. doi:10.1016/j.chest.2020.04.047
Galioto F, Palmucci S, Astuti GM, et al.Complications in idiopathic pulmonary fibrosis: focus on their clinical and radiological features.Diagnostics. 2020;10(7):450. doi:10.3390/diagnostics10070450
Perelas A, Silver RM, Arrosi AV, Highland KB.Systemic sclerosis-associated interstitial lung disease.Lancet. 2020;8(3):304-320. doi:10.1016/S2213-2600(19)30480-1
Chen X, Guo J, Yu D, Jie B, Zhou Y.Predictors of mortality in progressive fibrosing interstitial lung diseases.Front Pharmacol. October 2021;12:754851. doi:10.3389/fphar.2021.754851
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