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Updated: Feb 12 2018


  • Overview
    • Amyloid is misfolded protein that takes the form of a beta-pleated sheet
      • the protein cannot be degraded by cellular enzymes
      • results in accumulation in the extracellular space
      • the deposited mass of the misfolded protein is damaging to tissues
    • Diagnosis made by demonstrating
      • apple-green birefringence of Congo red stain under polarized light
    • Amyloid can form from a variety of proteins but the above characterisitic are shared properties
    • Amyloid can be localized or systemic
  • Systemic Amyloidosis
    • Affects the entire body and can be of primary or secondary cause
    • Primary
      • result of AL amyloid deposition
        • derived from Ig light chain
        • classically seen in multiple myeloma
    • Secondary
      • result of AA amyloid deposition
        • derived from acute phase reaction of inflammation
          • associated with increased levels of IL-6
        • can see with rheumatoid arthritis or other forms of chronic inflammation
    • Clinical findings
      • deposition in glomerulus
        • results in nephrotic syndrome
      • deposition in the heart
        • results in arrhythmias and/or restrictive cardiomyopathy
      • deposition in liver, spleen, tongue, intestine
  • Localized Amyloidosis
    • Affects only a specific organ
      • Localized Amyloidosis
      • Types
      • Protein
      • Derived from
      • Senile cardiac
      • AF
      • Transthyretin
      • Isolated Atrial
      • AANF
      • Atrial Natriuretic Peptide (ANP)
      • Diabetes mellitus type 2
      • AE
      • Amylin
      • Medullary carcinoma of the thyroid
      • A-CAL
      • Calcitonin
      • Alzheimer's disease
      • β-amyloid
      • Amyloid precursor protein (APP)
      • Dialysis-associated
      • β2-microglobulin
      • MHC class I proteins
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