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Interstitial Lung Disease: What Patients Need to Know

Overview Of Interstitial Lung Disease (ILD)

  1. What Is Interstitial Lung Disease (ILD)
    1.1 Understanding How ILD Affects The Lung Interstitium
  2. Common Symptoms Of Interstitial Lung Disease (ILD)
  3. What Causes Interstitial Lung Disease (ILD)?
  4. Complication and Long-term Effect Of Interstitial Lung Disease (ILD)
  5. Diagnosis and Treatment Options
    5.1 Diagnostic For ILD
    5.2 Possible Treatments For ILD

What Is Interstitial Lung Disease?

Interstitial lung disease (ILD), also known as Diffuse Parenchymal Lung Disease. Is a various of conditions that damage and scar to the lung tissues around the alveoli – the ‘air sacs’ of the lungs. These conditions affect the interstitium and make breathing harder over time.

The Illustration showing Interstitial Lung Disease. Image Credited by: ILD Collaborative

Understanding How ILD Affects The Lung Interstitium

The lung interstitium exists in a delicate space between your lungs’ air sacs (alveoli) and blood vessels. Connective tissue in this space supports your lungs’ structure. Oxygen passes through your air sacs, moves across the interstitium, and enters your bloodstream during normal breathing. Your blood releases carbon dioxide that travels through the interstitium and into your air sacs before you exhale it.

Your lung interstitium becomes thick and stiff as ILD develops. The thickened tissue creates a barrier that blocks oxygen from moving into your bloodstream and carbon dioxide from leaving your body. Your body gets less oxygen, so you feel short of breath, especially during physical activity.

The disease makes oxygen transfer more difficult as time passes. Doctors cannot reverse the scarring, and your lungs’ ability to work properly continues to decline. People with advanced ILD often need supplemental oxygen because their blood oxygen levels become too low.

If you have an Interstitial Lung Disease, other compartments of your lungs can also be affected, including:

  • Alveoli
  • Airways (trachea, bronchi, and bronchioles)
  • Blood vessels
  • Pleura (outside lining of the lung).

Types Of Interstitial Lung Disease (ILD)

ILDs have several broad categories based on their causes:

  • Idiopathic ILDs: Doctors cannot find the cause of these diseases. Idiopathic pulmonary fibrosis (IPF) leads this category as the most common type. “Idiopathic” means the cause remains unknown despite medical testing.

  • Occupational ILDs: Long-term exposure to harmful workplace substances causes these conditions. Common examples include asbestosis from asbestos exposure, silicosis from silica dust, and coal worker’s pneumoconiosis.

  • Autoimmune-related ILDs: Your immune system attacks your lungs in these cases. Rheumatoid arthritis, scleroderma, and lupus often trigger these conditions.

  • Medication-induced ILDs: Some medications can cause lung inflammation, especially certain chemotherapy drugs and heart medications.

It is estimated to affect more than 80,000 people annually in Malaysia. Some forms take years or decades to progress, but others can advance faster and cause severe breathing problems within months.

Common Symptoms Of Interstitial Lung Disease (ILD)

Your body sends warning signs when interstitial lung disease develops. These signs help you get treatment before the condition becomes severe. ILD shows a unique pattern of symptoms that emerge as lung damage increases.

Shortness of breath and dry cough

Shortness of breath stands out as the earliest and most common sign of interstitial lung disease. You might notice breathing difficulties when climbing stairs or exercising at first. Simple activities become challenging as ILD reduces your lungs’ oxygen transfer to your bloodstream. This symptom affects almost every ILD patient.

A persistent dry cough troubles up to 80% of people with idiopathic pulmonary fibrosis. The cough feels “hacking” or irritating because it doesn’t produce phlegm or mucus. Standard cough medications often fail to control this bothersome symptom effectively.

Fatigue and chest discomfort

ILD brings an overwhelming tiredness unlike regular fatigue. Rest doesn’t help this profound exhaustion that affects daily activities substantially. Research shows fatigue affects up to 95% of patients with idiopathic pulmonary fibrosis and 90% of those with sarcoidosis. Work performance suffers so much that 43% of sarcoidosis patients need disability evaluation.

Chest discomfort commonly accompanies ILD. Patients describe feeling tightness, pressure, or pain that gets worse with breathing. Advanced stages of the disease often bring appetite loss and unexplained weight changes.

Progression of symptoms overtime

ILD symptoms begin quietly and worsen over time. Mild breathlessness during exercise can turn into labored breathing even during talking or eating. Your body might need more oxygen as the disease advances.

The disease progression brings additional signs:

  • Rapid and shallow labored breathing
  • Daily activities become harder to complete
  • Advanced cases show blue discoloration of lips or fingernails (cyanosis)

Permanent lung damage often exists by the time symptoms become obvious. This reality emphasizes why early medical care matters so much. Click on the button if you need professional consultation.

What Causes Interstitial Lung Disease (ILD)?

Several factors can damage your lung tissue and lead to interstitial lung disease. You and your doctor can create a better treatment plan by knowing why ILD happens.

Environmental and occupational exposure

Industrial settings expose millions of workers to substances that cause ILD. Your lungs can suffer damage from continuous or repeated contact with certain toxins when you don’t wear protective gear. These harmful substances include:

  • Silica dust from construction, mining, or stonecutting
  • Asbestos fibers once commonly used in building materials
  • Wood, coal, and grain dusts
  • Mold and animal droppings

Autoimmune and connective tissue disease

Sometimes your body’s immune system attacks your lungs and causes inflammation and scarring. People commonly develop ILD with these autoimmune conditions:

  • Rheumatoid arthritis
  • Scleroderma (affects about 35-45% of patients)
  • Lupus
  • Dermatomyositis and polymyositis

Idiopathic pulmonary fibrosis and other unknown causes

Doctors can’t find the cause in many ILD cases. These cases are called idiopathic, with idiopathic pulmonary fibrosis (IPF) being the most common type. Risk factors for IPF include:

  • Age (most common in people 60-70 years old)
  • Male gender (more common in men)
  • Smoking history
  • Family history (genetic factors)

Complication and Long-term Effect Of Interstitial Lung Disease (ILD)

As interstitial lung disease progresses, you might face several serious complications that affect your quality of life and overall health. 

  • Pulmonary hypertension
  • Heart strain
  • Respiratory failure
  • Oxygen deprivation
  • Lung infection
  • Lung cancer

A clear understanding of what it all means helps you better prepare for the future and shows why early treatment matters.

Diagnosis and Treatment Options

Diagnostic For ILD:

  • Blood Test – Help detect markers of autoimmune conditions or environmental exposures that may contribute to ILD.
  • Chest X-ray – This imaging technique can reveal subtle patterns, such as fine lines, indicating lung abnormalities.
  • CT Scan – A high-resolution CT scan provides detailed images of lung tissue, helping confirm ILD and distinguish between its various forms.
  • Spirometry – A pulmonary function test that evaluates how much air your lungs can hold and how efficiently you can exhale.
  • Endobronchial Ultrasound (EBUS) – Biopsy a small sample of lung tissue may be collected to examine for inflammation, scarring, or other disease-specific changes.

Possible Treatments For ILD:

  • Medication
  • Oxygen therapy
  • Pulmonary Rehabilitation

There is no ‘one-size-fits-all’ treatment for it.  

Instead, treatment will vary depending on your condition’s suspected cause and severity. And since most lung damage is irreversible, it is best to focus treatment on preventing any more scarring, managing your symptoms and maintaining your quality of life by keeping you as healthy and active as possible.  Consult our Lung Specialist for a treatment that suits you the most.

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