What Is Interstitial Lung Disease?

Interstitial lung disease is an umbrella term for approximately 200 relatively rare lung diseases that cause inflammation or scarring in lung tissue, including connective tissue, air sac walls and spaces around blood vessels and airways. Asbestos exposure is one known cause of ILD, and asbestosis is the most well-known asbestos-related form. Some ILD types cause only scarring (fibrosis), some cause only inflammation and others cause both.

Key Facts About Interstitial Lung Disease

  1. Genetics, asbestos exposure, lifestyle factors, medications and other health conditions can cause interstitial lung disease.
  2. ILD covers diseases that scar the lungs.
  3. Symptoms may take years to develop. They can cause coughing, fatigue and shortness of breath.
  4. You can’t reverse lung damage, but early treatment can slow or stop its progression.

If you have inflammation without scarring, early treatment may prevent scarring from developing. Inflammation and scarring affect the interstitial lining that supports your lung’s alveoli. Your interstitium is a thin, mesh-like network of tissue that surrounds and supports the tiny air sacs in your lungs. When this tissue becomes thickened or scarred, it makes it harder for oxygen to pass into the bloodstream.

Types of Interstitial Lung Disease

Doctors usually group ILDs into broad categories according to their cause. These categories include exposure to irritants, some types of medical treatment, autoimmune disorders and smoking. 

The causes of some ILD cases can’t easily be identified. These are often called idiopathic pulmonary fibrosis. Idiopathic means the disease was spontaneous or the cause is unknown.

Types of ILDs

  • Desquamative interstitial pneumonitis: Cigarette smoking can cause many serious, fatal illnesses. It’s responsible for this form of ILD.
  • Hypersensitivity pneumonitis: This type of ILD comes from inhaling irritants like dust or mold.
  • Idiopathic pulmonary fibrosis: This is a chronic and progressive fibrosis from an unknown cause.
  • Interstitial pneumonia: Bacteria, fungi or virus exposure can cause an often temporary condition with inflammation of the interstitium.
  • Sarcoidosis: An inflammatory disease that mainly affects the lungs and lymph nodes. It can also affect other organs.

Risks include exposure to asbestos, silica dust and cotton dust. Asbestosis develops when the body tries to expel inhaled asbestos fibers, triggering inflammation and scarring. It’s a progressive disease that continues to worsen over time.


Autoimmune disorders contributing to ILD include lupus, polymyositis or dermatomyositis, rheumatoid arthritis and scleroderma.  Some medical treatments may cause lung inflammation and scarring. These treatments include chemotherapy, radiation and certain medicines.

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Interstitial Lung Disease Symptoms

Many of the different forms of ILD display similar symptoms associated with decreased lung function. They usually appear after scarring develops. This means that irreversible damage has occurred.

Symptoms of asbestosis can easily be mistaken for signs of other lung conditions such as COPD or asthma. Many ILD symptoms worsen with exertion.

Interstitial Lung Disease Symptoms

  • Chest pain or discomfort
  • Clubbed fingers
  • Difficulty or labored breathing
  • Dry cough
  • Fatigue and weakness
  • Fever
  • Shortness of breath
  • Unexplained loss of appetite or weight

Latency periods vary among the types of interstitial lung disease. Symptoms can take months to years to appear, depending on the disease. Some ILDs have a long latency period, so symptoms may not appear right away. Others advance more rapidly. 

It can take 20 to 30 years for asbestosis symptoms to develop. Some people have no symptoms at all. Many people don’t know they have the condition until lung damage becomes significant many years after exposure.

Complications

People with ILD can suffer from serious complications. Many ILD complications result from decreased lung function and its impact on the body. They include fluid and calcium buildup in the lung lining, high blood pressure in the lungs and a higher risk of infection and some cancers.

Interstitial Lung Disease Complications

  • Collapsed lungs
  • Lung infections
  • Pleural effusions
  • Pleural plaques
  • Pulmonary hypertension
  • Venous thromboembolism

Lung cancer is a big concern for people with asbestosis who smoke. Quitting smoking can lower your cancer risk and boost lung function.

Additionally, acute exacerbation, a sudden and severe worsening of breathing symptoms, is a serious concern. This can happen at any time and for many people, it’s what typically leads to a diagnosis.

Jerry Cochran aboard a Navy aircraft carrier

Jerry Cochran

Verified Asbestos.com Survivor

Navy Veteran With Asbestosis & Silicosis Has a Passion for Justice

Navy veteran Jerry Cochran was diagnosed 50 years ago with sarcoidosis. The more he read about it, the more concerned he was that his symptoms just didn’t match up. In 1991, he was given a new diagnosis – silicosis – from inhaling silica dust while scraping paint on the USS Independence. He was also later diagnosed with asbestosis, an asbestos-related interstitial lung disease.

Jerry Cochran

Verified Asbestos.com Survivor
Read Jerry’s Story

Interstitial Lung Disease Diagnosis

Doctors diagnose interstitial lung diseases using chest X-rays, CT scans and lung function tests. When asbestos-related ILD is suspected, doctors also ask about a person’s work and exposure history, since asbestos disease can take decades to appear.

Because ILDs share symptoms with other lung problems, diagnosis can be challenging. Detailed testing helps rule out other conditions, allowing doctors to make an accurate diagnosis. A high-resolution CT scan shows fine lung detail and can reveal the extent and pattern of lung scarring, helping separate it from other lung conditions.

Sometimes, a lung biopsy is needed. Tissue samples show lung health and may uncover other issues like cancer. Doctors collect these samples through procedures such as bronchoscopy, lavage or biopsy.

Diagnostic Bronchoscopy for Interstitial Lung Disease
Diagnostic Bronchoscopy for Interstitial Lung Disease
Bronchoscopy is often used to diagnose interstitial lung diseases.

Treatment for Interstitial Lung Diseases

Treatment for ILD and asbestosis focuses on easing symptoms, managing complications and slowing lung scarring. Oxygen therapy can reduce shortness of breath associated with lung damage. 

Doctors often prescribe medications including corticosteroids and anti-fibrotic drugs, which may relieve symptoms and slow the scarring process. For pulmonary fibrosis, anti-fibrotic options include Ofev (nintedanib) and Esbriet (pirfenidone).

ILD & Asbestosis Treatment Options

  • Lifestyle changes
  • Oxygen therapy
  • Prescription medications
  • Pulmonary rehabilitation

More advanced treatments include removing fluid from the lungs and respiratory therapy. Respiratory therapists teach breathing exercises to help people improve lung function. In rare cases, a lung transplant may be necessary, though it’s not a common treatment for asbestosis.

The outlook after an ILD diagnosis varies widely depending on the specific disease, overall health and lifestyle factors. Smoking is a significant risk factor, particularly for people with asbestos exposure. For asbestosis specifically, prognosis depends on the length and severity of exposure, and many people live full lives for years after diagnosis.

“For asbestosis, like pulmonary fibrosis, there aren’t great treatment options for this type of scar tissue… It’s treated symptomatically, which means there are options to reduce the amount of symptoms a patient has, such as breathing treatments, oxygen, or pain management.”

sean marchese
Sean Marchese , MS, RN and oncology writer

Frequently Asked Questions

Can asbestos cause pneumonia?

Asbestos exposure doesn’t directly cause infectious pneumonia, but it can trigger non-infectious lung inflammation that falls under the ILD umbrella. People with asbestos-related lung scarring also face a higher risk of lung infections, including pneumonia, because their lungs are already damaged.

Can asbestos cause sarcoidosis?

Sarcoidosis is an inflammatory ILD whose cause isn’t fully understood and researchers haven’t established a direct link to asbestos. Because sarcoidosis and asbestos-related ILD can look similar on imaging and share symptoms, doctors rely on a careful thorough testing to tell them apart.

What lung diseases can asbestos cause?

Asbestos exposure is linked to many asbestos-related diseases including asbestosis, pleural plaques, pleural thickening, pleural effusions and cancers such as lung cancer and mesothelioma.

Is asbestosis the same as interstitial lung disease?

Asbestosis is a type of ILD, which is the broad umbrella term for a large group of lung conditions.

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