Interstitial Lung Disease

Interstitial Lung Disease & Pulmonary Fibrosis

Illustration of the lungs and airways

Interstitial lung disease (ILD) is a group of conditions that cause inflammation or scarring (fibrosis) of the lung tissue, making it harder for oxygen to pass into the blood. Causes include autoimmune diseases such as rheumatoid arthritis and scleroderma, exposures to dusts, molds or birds, certain medications and radiation. When no cause is found and scarring progresses, it may be idiopathic pulmonary fibrosis (IPF).

Symptoms include:

  • Gradually worsening shortness of breath
  • A dry, persistent cough
  • Fatigue
  • Crackles heard in the lungs
  • Clubbing (rounding) of the fingertips
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Diagnosis & Treatment

How we diagnose ILD

  • High-resolution CT of the chest
  • Pulmonary function tests, including diffusion capacity, and a 6-minute walk test
  • Blood tests for autoimmune disease and a detailed exposure history
  • Bronchoscopy or lung biopsy in some cases

Treatment options

  • Antifibrotic medications that slow lung scarring in IPF and other progressive fibrosis
  • Anti-inflammatory or immune-suppressing medications for inflammatory types of ILD
  • Removing exposures that drive the disease
  • Oxygen and pulmonary rehabilitation
  • Vaccines, regular monitoring, and referral for lung transplant evaluation when appropriate

See also: Sarcoidosis · Pulmonary Hypertension

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