TRACHEAL AMYLOIDOSIS
 
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TRACHEAL AMYLOIDOSIS

 

DEFINITION:

A disease of the trachea caused by primary amyloidosis characterized by cough, dyspnea, and wheezing.

EPIDEMIOLOGY:

  • incidence: rare
  • age of onset:
    • childhood
  • risk factors:
    • ?

PATHOGENESIS:

1. Background

  • primary amyloidosis -> progressive deposition of amyloid in the tracheal wall -> gradual reduction in the tracheal lumen -> respiratory difficulties

CLINICAL FEATURES:

1. Respiratory Manifestations

  • 1. Early
    • cough
    • dyspnea
    • wheeze
  • 2. Late
    • cough, dyspnea, wheeze
    • hemoptysis
    • recurrent respiratory infections
    • respiratory distress

    • INVESTIGATIONS:

    1. Imaging Studies

  • 1. Chest X-ray
    • normal
  • 2. Bronchoscopy
    • diagnostic and therapeutic
    • narrowed tracheal lumen with friable tissue
    • biopsy: amyloid deposition in lumen

    • MANAGEMENT:

    1. Bronchoscopy

    • for removal of amyloid from tracheal lumen
    • may need to be repeated

     

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