Interstitial Lung Disease

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Interstitial lung disease (ILD) is a term used to describe a group of lung disorders that affect the tissue and space surrounding the air sacs of the lungs (a.k.a. the interstitium), causing lung scarring. The interstitium is responsible for exchanging oxygen and carbon dioxide between the lungs and the bloodstream.

In ILD, the interstitium becomes inflamed and/or subject to lung scarring, which can lead to difficulty breathing and decreased oxygen levels in the body. ILD can be caused by a variety of factors, including environmental and occupational exposures, genetics, and autoimmune disorders. 

Symptoms can vary depending on the specific type of ILD, but common ones include shortness of breath, cough, fatigue, and chest pain.

Diagnosis typically involves imaging tests such as X-rays or CT scans along with pulmonary function tests to measure how well your lungs are working

What are examples of interstitial lung disease?

Idiopathic pulmonary fibrosis (IPF)

This is a progressive, irreversible illness in which the lung tissue becomes thickened, stiff, and scarred, making it difficult to breathe.


This is a disease that causes inflammation in various organs, including the lungs. In the lungs, it can cause the formation of small clusters of inflammatory cells (granulomas) that can interfere with lung function.

Hypersensitivity pneumonitis (HP)

This is an allergic reaction to inhaled substances such as dust, mould, or animal dander, which can cause inflammation and damage to the lung tissue.

Connective tissue disease-associated interstitial lung disease

This is a group of lung diseases that occur in people with connective tissue diseases such as rheumatoid arthritis, systemic lupus erythematosus, and scleroderma.

Pulmonary fibrosis associated with other conditions

This is a group of lung diseases that occur in people with other underlying medical conditions such as asbestosis, silicosis, and drug-induced pulmonary fibrosis.

Acute interstitial pneumonia

This is a rapidly progressing form of ILD that can be life-threatening.

Non-specific interstitial pneumonia (NSIP)

This is a type of ILD that causes inflammation and scarring in the lung tissue but without the granulomas seen in sarcoidosis.

Who is most at risk for interstitial lung disease?

Some of the known risk factors for ILD include the following;

Environmental and occupational exposures

Exposure to certain substances in the workplace or environment can increase the risk of developing ILD. These substances may include silica, asbestos, coal dust, and grain dust.


Smoking is a major risk factor for many lung diseases, including ILD.


Some forms of ILD are believed to have a genetic component, meaning that certain genes may increase a person’s risk of developing the condition.


ILD is more common in older adults, and the risk increases as a person ages.

Autoimmune disorders

Certain autoimmune disorders, such as rheumatoid arthritis and scleroderma, are associated with an increased risk of ILD.

Gastroesophageal reflux disease (GERD)

There is some evidence to suggest that GERD may increase the risk of developing ILD.

It’s important to note that having one or more of these risk factors does not necessarily mean that a person will develop ILD. Additionally, some people may develop ILD without any known risk factors.

What causes interstitial lung disease?

The causes of ILD are not always clear, and in many cases, the exact cause is unknown. However, research suggests that a combination of genetic and environmental factors may contribute to the development of ILD such as pulmonary fibrosis.

  • Exposure to toxins, such as asbestos, silica, and bird droppings, can lead to ILD.
  • Certain infections, such as pneumonia, can damage lung tissue and lead to ILD.
  • Some medications, such as chemotherapy drugs and antibiotics, can cause ILD.
  • Certain autoimmune disorders, such as rheumatoid arthritis and scleroderma, can cause inflammation in the lungs and lead to ILD.
  • Some types of ILD are inherited and can be caused by genetic mutations.
  • In many cases, the cause of ILD is unknown, and it is referred to as idiopathic pulmonary fibrosis (IPF).

What are the symptoms of interstitial lung disease?

ILD can cause a range of symptoms that may develop gradually or suddenly, caused by factors such as lung scarring. These symptoms may vary depending on the underlying cause and the severity of the disease. 

Some common symptoms of ILD such as pulmonary fibrosis include:

  • Shortness of breath, especially during physical activity
  • Dry cough that persists for more than eight weeks
  • Chest pain or discomfort
  • Fatigue or weakness
  • Unexplained weight loss
  • Clubbing of the fingers (widening and rounding of fingertips)
  • Rapid, shallow breathing
  • Wheezing or crackling sound in the lungs
  • Loss of appetite
  • Swelling in the legs

It’s important to note that these symptoms can also be associated with other respiratory conditions, so it’s crucial to get a proper diagnosis from a healthcare professional if you are experiencing any of these symptoms.

What are the complications of interstitial lung disease?

Some possible complications of ILD include:

  • Pulmonary hypertension, such as ILD, can cause increased pressure in the blood vessels that supply the lungs, leading to pulmonary hypertension.
  • Respiratory failure because, in severe cases of ILD, the lungs may not be able to provide enough oxygen to the body, leading to respiratory failure.
  • Lung cancer is possible through some types of severe ILD, such as idiopathic pulmonary fibrosis (IPF).
  • Cor pulmonale, is a condition in which the right side of the heart becomes enlarged due to long-term high blood pressure in the lungs caused by ILD.
  • Infections, including pneumonia, due to the weakened state of their lungs.
  • Reduced quality of life, because ILD can significantly impact a person’s ability to breathe and perform daily activities.

How is interstitial lung disease diagnosed?

The diagnosis of interstitial lung disease typically involves a combination of tests and evaluations to determine the cause of the symptoms and the extent of the lung damage. Here are some of the common methods used to diagnose ILD such as pulmonary fibrosis;

Medical history and physical exam

The doctor will review the patient’s medical history and symptoms and perform a physical exam to look for signs of ILD, such as crackling sounds in the lungs or clubbing of the fingers.

Pulmonary function tests

These tests measure how well the lungs are functioning, including how much air the lungs can hold and how easily air can move in and out of the lungs. This helps in the diagnosis of pulmonary diseases such as pulmonary fibrosis.

Imaging tests

X-rays, CT scans, and MRIs can help identify abnormalities in the lungs, such as scarring or inflammation.


This procedure involves passing a thin, flexible tube with a camera through the nose or mouth and into the lungs to examine the airways and collect tissue samples for further testing.


A lung biopsy may be performed to remove a small sample of lung tissue for examination under a microscope. This can help determine the type of ILD and guide treatment.

Blood tests

Blood tests can help rule out other conditions that may cause similar symptoms.

High-resolution computed tomography (HRCT)

This type of CT scan uses thinner slices and more advanced imaging techniques to provide a more detailed look at the lungs and can help distinguish between different types of ILD.

It’s important for patients to work closely with a doctor who specialises in ILD to ensure an accurate diagnosis and appropriate treatment plan.

What kinds of respiratory tests help to potentially identify interstitial lung disease?

Manse Medical conducts the following respiratory tests to aid in the identification of conditions such as those associated with ILD;

Gas Transfer

This test measures how well oxygen moves from the lungs into the bloodstream. It involves breathing in a small amount of a harmless gas, such as carbon monoxide, and then exhaling it to measure how much was absorbed into the bloodstream.

Lung Function Testing

This broad term refers to a variety of tests that assess how well the lungs are functioning. It may include spirometry, lung volumes, gas transfer, and other tests.

Lung Volumes

This test measures the amount of air in the lungs, including the amount of air that can be inhaled and exhaled.


This test measures how much air a person can inhale and exhale, and how quickly they can do so. It can be used to diagnose and monitor conditions such as asthma, pulmonary fibrosis and chronic obstructive pulmonary disease.

Six-Minute Walk Test

This test measures how far a person can walk in six minutes and is often used to assess the functional capacity of the lungs and heart. It can be used to monitor the progression of conditions such as ILD or heart failure.

How is interstitial lung disease treated?

The treatment of ILD depends on the specific type of ILD, its severity, and the underlying cause. Here are some of the common treatments used for ILD;


Corticosteroids and immunosuppressant drugs may be used to reduce inflammation in the lungs and slow the progression of ILD. Other medications, such as antibiotics or antifungal drugs, may be prescribed if an infection is present.

Oxygen therapy

Supplemental oxygen may be prescribed to help improve oxygen levels in the blood and alleviate shortness of breath.

Pulmonary rehabilitation

A program of exercise, breathing techniques, and education can help improve lung function and quality of life for people with ILD.

Lung transplantation

For severe cases of ILD, lung transplantation may be an option.

Management of underlying conditions

If ILD is caused by an underlying condition, such as an autoimmune disease, treating that condition may help improve ILD symptoms.

It’s important to work closely with a doctor who specialises in ILD to develop an individualised treatment plan. In some cases, ILD may be managed with a combination of treatments, and ongoing monitoring may be necessary to assess the progression of the disease and adjust the treatment plan as needed.

How can I manage my interstitial lung disease symptoms?

There are several things you can do to help alleviate your symptoms and improve your quality of life, including;

  • If you smoke, quitting is the most important thing you can do to improve your lung health and slow the progression of ILD.
  • Exercise can help improve lung function and reduce shortness of breath. Talk to your doctor about the types and intensity of exercise that are safe and appropriate for you.
  • A well-balanced diet can help you maintain a healthy weight and provide your body with the nutrients it needs to function properly.
  • Stress and anxiety can worsen symptoms of ILD. Techniques such as deep breathing, meditation, and yoga may help you relax and manage stress.
  • Respiratory infections such as the flu and pneumonia can be particularly dangerous for people with ILD. Make sure you are up to date on your vaccinations and talk to your doctor about getting the flu and pneumonia vaccines.
  • It’s important to work closely with your doctor to develop an individualised treatment plan for your ILD. Make sure you understand your treatment plan and follow it closely, including taking any prescribed medications and attending any necessary appointments.
  • Pulmonary rehabilitation is a program of exercise, breathing techniques, and education that can help improve lung function and quality of life for people with ILD.



Interstitial Lung Disease FAQs

How is Interstitial Lung Disease treated?

Treatment approaches for ILD may include medications (such as corticosteroids or immunosuppressants), oxygen therapy, pulmonary rehabilitation, and in severe cases, lung transplantation. The choice of treatment depends on the specific type and severity of ILD.

Can Interstitial Lung Disease be prevented?

In some cases, ILD can be prevented by avoiding exposure to known causes, such as occupational hazards like asbestos or silica dust. Quitting smoking can also reduce the risk of developing ILD.

What is the prognosis for Interstitial Lung Disease?

The prognosis varies depending on the type and stage of ILD. Some forms may progress slowly, while others can be more aggressive. Early diagnosis and appropriate management can positively impact the prognosis and quality of life for individuals with ILD. Regular follow-ups with healthcare providers are essential for monitoring and adjusting treatment plans.

Is Interstitial Lung Disease contagious?

No, ILD is not contagious. It is not caused by infection or exposure to infectious agents, and it cannot be transmitted from person to person.

How quickly does Interstitial Lung Disease progress?

The progression of ILD varies widely among individuals. Some may experience a slow decline in lung function over several years, while others may face a more rapid deterioration. Regular monitoring by healthcare professionals is crucial for tracking progression.

What role do genetics play in Interstitial Lung Disease?

While some forms of ILD have a genetic component, most cases are not directly inherited. However, a family history of ILD or other lung diseases may increase the risk for certain individuals.

Can Interstitial Lung Disease lead to respiratory failure?

In advanced stages, ILD can lead to respiratory failure, where the lungs are unable to provide adequate oxygen to the body. This is a serious complication that may require advanced medical interventions, including mechanical ventilation or lung transplantation.

Does Interstitial Lung Disease increase the risk of other health problems?

ILD can increase the risk of developing complications such as pulmonary hypertension, heart problems, and infections. Managing ILD effectively with medical guidance can help mitigate these risks.

Where can I go for assessment for respiratory conditions such as those associated with interstitial lung disease?

At Manse Medical, we provide high-quality assessment, diagnosis and treatment for respiratory disorders such as interstitial lung disease. 

We take a personalised approach that focuses on your medical history and background, in order to provide you with a complete treatment plan that addresses your concerns and helps you effectively manage your symptoms.

Book your appointment online by selecting your preferred clinic and choosing from the list of available specialists. 

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