How Interstitial Lung Disease Affects Breathing: A Complete Guide

Interstitial Lung Disease (ILD) refers to a group of over 200 chronic lung disorders that cause inflammation and scarring (fibrosis) of the interstitium. It has devastating consequences on a person’s breathing function and quality of life.

This blog will tell you all you need to know about ILD, its causes, its symptoms, and how it is diagnosed and treated.

What is interstitial lung disease (ILD)?

Interstitial lung disease (ILD)  is a condition that affects the air sacs within the lungs, causing them to become inflamed, thickened, and scarred.

According to a recent study on the Global Burden of Diseases, the global incidence of ILDs has increased by 51% over the last decade. ILD is also a significant health concern in India, with an estimated prevalence that approximately ranges from 49 to 98 per 100,000 persons, though this number may be higher due to underdiagnosis.

ILDs are usually treated by a specialized pulmonologist. 

What are the causes of interstitial lung disease?

The exact cause of ILD is often unknown, but various factors may contribute, including:

• Drugs – certain chemotherapy drugs, heart medications, antibiotics, and anti-inflammatories can damage the lungs
• Infections – bacteria, viruses, or fungi can infect the interstitium
• Regular exposures to inhaled irritants – asbestos, silica dust, talc, coal, metal dust, and grain dust can cause some forms of interstitial lung disease
• Smoking – smoking or vaping is a significant contributing factor 
• Certain connective tissue diseases and autoimmune conditions – ILD is more common in this population 
• Unknown causes (Idiopathic) – the causes of most interstitial lung diseases have not yet been identified.

Who is affected by Interstitial Lung Disease?

• Older individuals: The highest rate of interstitial lung disease occurs in people aged 80 to 84 years. 
• Occupational risks: Jobs in mining, farming, or construction put the worker at high risk of developing ILD.
• Autoimmune conditions: Those having certain autoimmune conditions, such as rheumatoid arthritis, scleroderma, or systemic lupus erythematosus, commonly develop ILD as an associated disease.
• Female gender: Interstitial lung disease is more common in females, but idiopathic pulmonary fibrosis (IPF) affects males about 3 times more often.

How does Interstitial lung disease affect breathing?

The interstitium is the tiny, fluid-filled tissue and space around the air sacs in the lungs. In healthy people, the interstitium is very thin. On the other hand, in interstitial lung diseases, the interstitium thickens and scars. Over time, the scarring can cause lung stiffness and eventually affect breathing.

However, ILD typically affects not only the interstitium but also the airspaces, peripheral airways, arteries, and epithelial and endothelial linings of the lungs.

Symptoms of ILD That Affect Breathing

Symptoms of ILD can vary depending on the specific type of ILD.

Symptoms can include the following:

• The most common symptom of interstitial lung disease is shortness of breath which develops slowly, over several months. 
• Other symptoms include a dry, scratchy cough and unexplained weight loss.
• The person may also experience fatigue.
• Swelling and rounding of the fingertips (clubbing) may occur in some cases.

Patients with mild interstitial lung disease may not experience any noticeable symptoms for some time and often aren’t aware of the changes in how their lungs function.

How Doctors Diagnose ILD

Diagnosis of interstitial lung disease is typically made based on symptoms, physical examination, and additional testing.

Doctors diagnose ILD on the basis of one or more of the following tests:

• Physical examination – including listening to your lungs
• Blood tests – to identify specific causes of interstitial lung disease
• Chest X-ray – images in people with interstitial lung disease may show fine lines in the lungs
• High resolution CT scan – will identify if interstitial lung disease is present and help differentiate between different types of disease 
• Lung biopsy – a small amount of lung tissue is examined under a microscope to determine the interstitial lung disease diagnosis
• Oximetry – a test measuring the oxygen saturation in your blood.
• Lung function tests – measure how your lungs are working and track the progress of your disease over time

Treatment Options & Management

The aim of all treatments is to slow or stop the scarring process and treat any signs and symptoms.

• Antibiotics – medicines that fight bacterial infections
• Antifibrotics – medications such as pirfenidone and nintedanib that can slow the disease progression in idiopathic pulmonary fibrosis
• Bronchodilators – inhaled medicines that help to open up your airways. If you are prescribed an inhaler (or puffer), it’s important to use it correctly so that your lungs can benefit from the medication. 
• Immunosuppression – medicines that reduce inflammation and swelling in your airways
• Oxygen therapy – Oxygen may be suggested for some patients who could benefit from additional oxygen to help with breathlessness, protect your organs and enhance your quality of life
• Pulmonary rehabilitation – Exercises to enhance lung function and overall health.
• Surgery – in severe cases, your doctor may suggest surgery to remove diseased lung tissue or replace a diseased lung with a healthy one (lung transplant).

What are the complications of interstitial lung disease?

If untreated, ILD can lead to several complications, such as:

• Respiratory failure: Due to inadequate oxygenation.
• Pulmonary hypertension: Increased blood pressure in the lungs’ arteries.
• Heart failure: Strain on the heart from reduced lung function.
• Pneumothorax: Also known as a collapsed lung, and happens when air escapes from the lungs.
• Acute exacerbations: Sudden worsening of symptoms, requiring urgent medical care.
• Lung cancer: A form of cancer that occurs when abnormal cells grow uncontrollably in the lungs.

Breathing Techniques That Help ILD Patients

Pursed lip breathing, diaphragmatic breathing, and yoga breathing are 3 breathing techniques that reduce breathlessness and consistently improve the quality of life in people with ILD.

1. Pursed Lip Breathing

1. This is a simple technique for slowing the breathing and bringing more air into the lungs. This can help the lungs work more effectively.
2. Technique: 
   • Sit straight or lie down in a relaxed position.
   • Inhale two seconds through your nose, filling the abdomen with air
   • Purse your lips, leaving only a small opening for air to pass through.
   • Breathe out slowly and exhale twice as long as you inhale.
   • Repeat these steps for 5-10 minutes.

Diaphragmatic Breathing

1. This technique involves breathing in slowly and deeply through the nose using the diaphragm with a minimum movement of the chest.
2. It is a deep breathing method.
3. Technique: 
   • Lie down on the bed or any comfortable surface.
   • One hand should be placed on the chest and the other hand on the belly. 
   • Breathing in slowly and deeply for 6 seconds through the nose with a minimum movement of the chest.
   • During breathing, ensure that the chest remains as still as possible and the stomach moves against the hand placed on the belly. 
   • Then exhale slowly for 6 seconds.
   • Repeat the technique for 3-5 minutes.

Yoga Breathing 

1. Yogic breathing involves manipulation of breath movement.
2. It is recommended for relaxation, stress management, and control of breathing.
3. Technique: 
   • Ujjayi Breath: This involves experiencing the conscious sensation of the breath touching the throat. This is a slow breath technique (2–4 breaths per minute).
   • Bhastrika Breath: In this, the air is rapidly inhaled and forcefully exhaled at a rate of 30 breaths per minute. 
   • Caution: Do not continue this breathing technique if you experience breathlessness while doing it.
   • Om chanting: “Om” is chanted three times while taking a very prolonged exhalation of air.

Useful Tips: 

• Adding breathing exercises to whole-body exercise training further improves breathlessness in people with ILD.
• Although the likelihood of adverse effects with breathing exercises is very low, it is recommended to do the breathing techniques after consulting with a health professional.

Frequently Asked Questions