amyloidosis

• extracellular deposition of insoluble B-pleated sheet configured protein that stains with Congo red

• systemic (80-90%)
  • primary
    • M > F
    • 55-60y
    • often presents with renal insufficiency or CHF
  • secondary
    • multiple myeloma
    • rheumatoid arthritis
    • familial Mediterranean fever
    • chronic infection (TB, syphillis)
• localized (10-20%)

manifestations

• chest
  • tracheal nodules/narrowing
  • pulmonary nodules
  • diffuse parenchymal pattern
• heart: enlargement (Tc-99m pyrophosphate positive)
• kidneys
  • large kidneys
  • small kidneys
  • focal masses
  • calcifications
• bladder/ureters: focal or diffuse infiltration
• bones: pain/weakness associated with lytic lesions +/- calcium
• joints (shoulder, elbow, wrist, hip)
  • ST enlargement
  • subchondral cysts
• gastrointestinal tract

© 2002, C.E. Kahn, Jr.
2002-04-18