Updated: 3/28/2019

Cryptogenic Organizing Pneumonia

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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
  • Prognosis
    • typically very good with treatment
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
  • Making the diagnosis
    • based on clinical presentation and studies
    • definitive diagnosis via biopsy results
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
Treatment
  • Management approach
    • COP does not respond to antibiotics
  • First-line
    • steroids
Complications
  • Relapse of disease
  • Respiratory failure
    • rare
 

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