Amyloidosis

• Facts:
  Extracellular deposition of protein fibril / amyloid proteins in the body's tissues and organs as beta pleated sheets
  Can be hereditary or acquired
  MC types of amyloidosis are AL and AA
• History / PE:
  Enlarged kidneys
  Hepatomegaly
  Proteinuria ( nephrotic syndrome )
  Easy bruisability
• Diagnosis:
  Tissue biopsy with congo red stain shows apple-green birefringence under polarized light
• Treatment:
  Chemotherapy
  Transplant
• Complications:
  Renal amyloidosis
  Restrictive cardiomyopathy ( diastolic dysfunction )
• Notes:
  Different kinds
  
  1.) Primary amyloidosis (AL)
  2.) Secondary amyloidosis (AA)
  3.) Dialysis related (deposition of beta 2 microglobulin)
  4.) Heritable
  5.) Senile-systemic
• Associated With:
  Rheumatoid arthritis
  Nephrotic syndrome
  Multiple myeloma
  Waldenstrom's macroglobulinemia