Friday, December 4, 2015

Amyloidosis

Epidemiology

  • Extracellular deposit of insoluble polymeric protein fibrils in tissue & organs
  • Can be primary (AL type) or secondary (AA) to chronic inflammatory conditions such as:
  • Inflammatory arthritis (e.g RA)
  • Chronic infections (e.g bronchiectasis, tuberculosis, osteomyelitis)
  • Inflammatory bowel disease (e.g Crohn's disease)
  • Malignancy (e.g. lymphoma)
  • Vasculitis

Clinical presentation

  • Asymptomatic proteinuria or nephrotic syndrome 
  • Restrictive cardiomyopathy
  • Hepatomegaly
  • Peripheral neuropathy &/or autonomic neuropathy
  • Visible organ enlargement (e.g. macroglossia)
  • Bleeding diathesis
  • Waxy thickening, easy bruising of skin

Diagnosis 

  • Tissue biopsy (e.g. abdominal fat pad)

Congo red stain shows amyloid deposits within vessel walls. 


Congo red stain shows apple green birefringence under polarized light.

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