Interstitial lung disease


Lobes of the lung with related anatomy.
  • General group of diseases that involve the scarring of lung parenchyma
  • Grouped together because of similar clinical, physiologic, radiographic, or pathologic findings
  • Specific diseases separated into ones with known causes and idiopathic
  • Treatment and prognosis differs between specific diseases
  • Examples of specific diseases:

Clinical Features

  • Symptoms
    • Progressive exertional dyspnea
    • Persistent, nonproductive cough
  • +/- hemoptysis, pleuritic chest pain
  • May have history of smoking, occupational exposures (asbestosis, silicosis, grain dust, bird droppings), medications that can cause pulmonary damage, connective tissue disease, IBD
  • Physical exam
    • Usually nonspecific
    • Increased work of breathing, hypoxia
    • +/- Abnormal lung sounds (classically fine, "velcro-like" crackles)

Differential Diagnosis

Pulmonary Fibrosis

Acute dyspnea




  • Definitive diagnosis of ILD rarely made in the ED
  • ED workup should focus on assessing for reversible pathologies that may be causing or exacerbating symptoms (e.g. infection, PE, pulmonary hypertension, pulmonary edema, concomitant COPD exacerbation, etc.)
    • VBG/ABG
    • CBC, BMP
    • CXR
    • Consider CT chest (to rule out PE or better characterize lung parenchyma)
    • +/- Infectious workup, echo as appropriate
  • Inpatient/outpatient workup may include serologic studies (ANA, rheumatoid factor), PFTs, BAL, lung biopsy


  • Treat underlying/exacerbating factors (e.g. antimicrobials for infection, optimize volume/hemodynamics for pulmonary hypertension patients, bronchodilators for concomitant obstructive lung disease)
  • Support oxygenation and ventilation
  • Specific disease treatments vary, but many respond to steroids, sometimes at high doses


  • Low threshold to admit

See Also