Written by Karen Selby, RN | Medically Reviewed By Dr. Rupesh Kotecha | Edited By Walter Pacheco | Last Update: June 20, 2024

What Is Interstitial Lung Disease?

Interstitial lung disease is an umbrella term for approximately 200 different relatively rare lung diseases that cause lung inflammation or scarring. Some ILD types only cause scarring (fibrosis), some only cause inflammation and others can cause both. 

In instances where a patient has inflammation without scarring, early treatment may prevent scarring. The inflammation and scarring affect the interstitial lining that supports the lung’s alveoli. This decreases lung function as thickened tissue can’t supply oxygen to the blood as effectively as healthy lung tissue.

Diagram of lungs showing bronchioles, alveoli and capillaries. Scar tissue has developed on the alveoli.
Interstitial lung disease causes scar tissue to form in the lungs after exposure to asbestos and other harmful materials.

Environmental factors, some health conditions, medications and genetic predisposition can contribute to the development of this disease. Asbestosis, for example, is an ILD linked to asbestos exposure. As the body tries to expel inhaled asbestos fibers, inflammation and scarring can occur. It’s a progressive disease that continues to worsen over time.  

Key Facts About Interstitial Lung Disease
  • Genetics, environmental and lifestyle factors, medications and other health conditions can cause interstitial lung disease.
  • ILD covers several diseases that cause lung scarring.
  • Symptoms can take years to develop and may include coughing, fatigue and shortness of breath.
  • You can’t reverse lung damage, but early treatment can slow or stop its progression.

Types of Interstitial Lung Disease

ILDs are typically grouped into broad categories related to their cause. These 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 classified as 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 and terminal illnesses and is responsible for this form of ILD.
  • Hypersensitivity pneumonitis: Repeatedly inhaling irritants, such as dust or mold, causes this type of ILD.
  • 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 usually affects the lungs and lymph nodes but can also affect other organs

Autoimmune disorders contributing to ILD include lupus, polymyositis or dermatomyositis, rheumatoid arthritis and scleroderma. Potential exposure risks include asbestos, silica dust and cotton dust. Medical treatments that may cause lung inflammation and scarring include chemotherapy, radiation and certain medicines.

Interstitial Lung Disease Symptoms

Many of the different forms of ILD display similar symptoms associated with decreased lung function. They typically appear after scarring develops, meaning that irreversible damage has already 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 different forms of interstitial lung disease. The onset of symptoms can take from months to years depending on the disease progression. Some ILDs have a long latency period, so symptoms may not appear right away, while others advance more rapidly. 

It can take 20 to 30 years for asbestosis symptoms to develop, and some patients don’t exhibit symptoms at all. Many patients don’t know they have the condition until lung damage becomes significant many years after exposure. 


Patients with ILD can experience several potentially serious complications. Many of them are the result of decreased lung function and how that affects the body. They include accumulations of fluids and calcium deposits in the lining of the lungs, high blood pressure within the lungs and increased risk of infection and certain types of cancer.

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

Lung cancer is a particular concern among patients with asbestosis who are also smokers. Quitting smoking can reduce your chances of developing cancer and improve overall lung function. 

Additionally, acute exacerbation is a serious concern. This sudden worsening of respiratory symptoms affects a large number of patients with ILD and can occur at any time. For many patients, it can be what ultimately leads to a diagnosis.

Survivor Story
Survivor Story
Jerry Cochran Asbestosis & Silicosis

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.

Read Jerry’s Story

Interstitial Lung Disease Diagnosis

Doctors use chest X-rays, CT scans and pulmonary function tests to diagnose interstitial lung diseases. The similarity of symptoms between ILDs and other lung conditions can make getting a diagnosis difficult. Comprehensive testing can help rule out other conditions, so doctors can make a proper diagnosis.

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

In some cases, a lung biopsy may be necessary. Tissue collected can show the condition of lung tissue and may detect the presence of other lung conditions, such as cancer. Doctors collect samples using a bronchoscopy, bronchoalveolar lavage or surgical biopsy.

Treatment for Interstitial Lung Diseases

ILD and asbestosis treatment focuses on relieving symptoms, reducing complications and slowing further scarring of the lungs. Oxygen can help address shortness of breath from existing damage. Other medications, including corticosteroids, anti-fibrotics and tyrosine kinase inhibitors, may help relieve some symptoms and slow disease progression. 

Asbestosis is a type of pulmonary fibrosis. A large amount of scarring in the lungs characterizes the condition. 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.

More intensive interventions include draining fluid from the lungs and respiratory therapy. In the latter, a trained therapist teaches patients breathing exercises to improve lung function. In rare cases, a lung transplant may be necessary, but this isn’t a common treatment for asbestosis.

ILD & Asbestosis Treatment Options
  • Lifestyle changes
  • Oxygen therapy
  • Prescription medications
  • Pulmonary rehabilitation

The prognosis after an ILD diagnosis varies tremendously according to disease type. General physical condition and certain lifestyle factors, such as smoking, can impact disease progression. Smokers exposed to asbestos have a high likelihood of developing ILD. 

With asbestosis, patient prognosis often depends on the length and severity of asbestos exposure. Individuals may live full lives for many years after a diagnosis.

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