Interstitial Lung Disease

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Interstitial lung diseases (ILD), also called pulmonary fibrosis, includes over 200 disorders that lead to inflammation and scarring. This is called fibrosis in the lungs.

This process can damage the air sacs of the lung where gas exchange occurs leading to symptoms of shortness of breath and cough, and poor oxygen uptake.

Specific types of interstitial lung disease include:

  • Idiopathic pulmonary fibrosis, a progressive lung scarring disease of unknown cause 
  • Connective tissue disease, when the immune system mistakenly attacks the lungs (like lupus, rheumatoid artheritis, scleroderma and dermatomyositis)
  • Hypersensitivity pneumonitis, in which the immune system mistakenly overreacts to inhaled particles (like reactions to farming environments, birds and fungal spores)
  • Sarcoidosis
  • Reactions to inhaled dusts (asbestosis, silicosis) or certain medications
  • Smoking-related interstitial lung diseases
  • Certain genetic illnesses

ILD Symptoms

The most common symptom of ILD is shortness of breath that worsens with time. Breathing problems occur with activity and at rest.

ILD may also cause:

  • Persistent, dry cough that can’t be controlled
  • Fatigue
  • Clubbing — nails that bulge or thicken

Initial Evaluation of Pulmonary Fibrosis

During your first visit, we obtain a detailed history of your illness. We also perform tests to assess the severity of the interstitial lung disease and to look for the cause of the disease. We will review prior tests from other hospitals as well. 

Tests for assessment of severity include:

    • Pulmonary function testing, to measure the extent of lung impairment
    • Assessment of oxygen levels when walking using pulse oximetry, to measure the need for supplemental oxygen therapy
    • Sometimes, echocardiography to assess heart function

Tests for assessment of cause of interstitial lung disease include:

    • Blood tests
    • High-resolution chest CT scans

After these results become available, we sometimes arrange to obtain biopsy samples from your lungs to examine under a microscope. Depending on the type of lung disease, we may obtain these samples using bronchoscopy or a surgical lung biopsy.

Do You Have a Pulmonary Fibrosis Diagnosis?

Learn about how we treat pulmonary fibrosis.