Updated: 12/24/2021

Cryptogenic Organizing Pneumonia

Review Topic
  • Snapshot
    • A 56-year-old man presents for a follow up with his primary care physician for a few weeks of low-grade fevers, cough, malaise, and shortness of breath. He had tested negative for tuberculosis and his sputum cultures had shown no growth. His symptoms have not responded to a course of azithromycin. His past medical history includes hypertension, for which he is taking hydrochlorothiazide. He has no history of autoimmune diseases. He currently works as a software engineer. On physical exam, there are sparse inspiratory crackles bilaterally. A chest radiograph shows bilateral patchy infiltrates, and he is sent for a high-resolution computed tomography.
  • Introduction
    • Clinical definition
      • cryptogenic organizing pneumonia (COP) is a rare organizing noninfectious pneumonia/bronchiolitis in which the cause is often unknown
        • may be caused by chronic inflammatory diseases or medications
      • previously known as bronchiolitis obliterans organizing pneumonia (BOOP)
  • Epidemiology
    • Incidence
      • rare
    • Demographics
      • adults
    • Risk factors
      • chronic inflammatory diseases
        • rheumatoid arthritis
        • other connective tissue diseases
      • medications
        • amiodarone
    • Pathogenesis
      • inflammation of the small airways (bronchioles) with chronic alveolitis
      • exact pathogenesis is unknown but thought to be related to alveolar injury and fibrotic alveolar bud formation
  • Presentation
    • Symptoms
      • systemic symptoms
        • fever
        • malaise
        • myalgia
      • cough
      • shortness of breath
    • Physical exam
      • respiratory
        • rales
        • inspiratory crackles
  • Imaging
    • Chest radiography
      • findings
        • bilateral patchy infiltrates
    • Chest computed tomography (CT)
      • findings
        • patchy and migratory ground-glass opacities
        • alveolitis
  • Studies
    • Sputum and blood cultures
      • negative
    • Transbronchial biopsy or video-assisted thoracoscopy
      • histology
        • endoluminal buds of granulation tissue and connective tissue
        • Masson body that plugs the small airways
        • obliterated airways
  • Differential
    • Pneumoconioses (e.g., asbestosis)
      • distinguishing factor
        • typically does not present with systemic symptoms
        • presents with a more chronic course
        • typically has a clear occupational exposure
        • does not respond to steroids
    • Making the diagnosis
      • based on clinical presentation and studies
      • definitive diagnosis via biopsy results
  • Treatment
    • Management approach
      • COP does not respond to antibiotics
    • First-line
      • steroids
  • Complications
    • Relapse of disease
    • Respiratory failure
      • rare
  • Prognosis
    • Typically very good with treatment

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