Interstitial Lung Disease

What Is Interstitial Lung Disease?

Interstitial Lung Disease (ILD) is a group of over 200 chronic lung disorders that cause inflammation and scarring (fibrosis) of the lung's interstitium (the tissue surrounding the lung’s air sacs). This scarring makes it harder for your lungs to work properly and can lead to difficulty breathing and reduced oxygen in the bloodstream.

Who Is Affected?

Interstitial lung disease can affect anyone, but it's more commonly diagnosed in adults over 50. Some types are linked to autoimmune conditions, environmental exposures, medications, or unknown causes (idiopathic). Both men and women are affected, though certain forms of interstitial lung disease may show a gender-related bias.

What Causes Interstitial Lung Disease?

Interstitial lung disease can be triggered by a wide variety of causes, or in some cases, have no identifiable origin (idiopathic). It generally develops due to lung tissue injury, followed by an abnormal healing response that causes scarring.

Non-Controllable Risk Factors

• Age
• Genetics
• Autoimmune conditions like rheumatoid arthritis, scleroderma, or lupus are linked to interstitial lung disease.
• Radiation therapy and some chemotherapy drugs can damage lung tissue.
• Certain viral and bacterial infections may trigger lung scarring.

Controllable Risk Factors

• Occupational Exposure: Long-term exposure to dust, asbestos, silica, or coal dust can lead to interstitial lung disease.
• Exposure to bird feathers
• Smoking
• Some heart medications, antibiotics, and anti-inflammatory drugs may cause lung damage.

Who Is Most at Risk?

People with autoimmune conditions, older adults, smokers, and individuals exposed to industrial pollutants or allergens are most at risk. Having a first-degree relative with interstitial lung disease also increases susceptibility.

How to Reduce Your Risk

• Quit smoking
• Wear protective gear in workplaces with harmful inhalants
• Regular health screenings if you have autoimmune conditions
• Inform your doctor about all medications you take

Common Symptoms of ILD

• Shortness of breath (especially during activity)
• Dry, persistent cough
• Fatigue or general weakness
• Chest discomfort
• Unintended weight loss
• Finger clubbing (widened and raised nail beds)

How Is Interstitial Lung Disease Diagnosed?

Interstitial lung disease is diagnosed through a combination of clinical evaluations, imaging, and lab tests. Early diagnosis can help slow disease progression.

Common Diagnostic Tests

• Chest X-Ray: Initial imaging to look for lung scarring.
• High-Resolution CT (HRCT) Scan: Detects the pattern and location of lung scarring.
• Pulmonary Function Tests (PFTs): Measure how well your lungs function.
• Blood Tests: Rule out autoimmune or infectious causes.
• Bronchoscopy: A small camera is used to examine lung tissue and obtain biopsies.
• Lung Biopsy: In rare or unclear cases, a surgical biopsy may be needed to confirm diagnosis.

There is no universal cure for interstitial lung disease, but treatments can help manage symptoms and slow disease progression.

Lifestyle

• Quit Smoking
• Pulmonary Rehabilitation
• Maintain a healthy lifestyle to support lung function

Medical Treatments

• Anti-inflammatory medications, such as prednisone
• Immunosppressive agents (in certain types of ILD), such as Azathioprine, Methotrexate
• Antifibrotic drugs such as nintedanib and pirfenidone to slow scarring progression
• Oxygen therapy

Surgical Treatments

• Lung Transplantation may be considered for eligible patients with advanced disease.

Assistive Devices

• Portable oxygen concentrators or home oxygen delivery systems can support daily activity and quality of life.

Other Resources

• Support Groups & Counseling: Can be vital for managing the emotional aspects of chronic lung disease.
• Vaccinations: Keeping up with flu, COVID-19, and pneumonia vaccines is critical.

When to Seek Care

When to Call Your Doctor

• Ongoing dry cough that doesn’t improve
• Shortness of breath during simple tasks
• Chronic fatigue or unexplained weight loss
  
  

When to Seek Emergency Medical Treatment

• Sudden chest pain
• Difficulty breathing or rapid breathing at rest
• Blue lips or fingertips (signs of low oxygen)
  
  

What Should You Do Next?

If you're experiencing symptoms or have risk factors for interstitial lung disease, talk to your primary care provider or a pulmonologist. Early diagnosis and treatment make a significant difference. You may be referred for lung imaging or pulmonary testing to evaluate your lung health further.