Amyloidosis Flashcards
describe what amyloidosis is
the deposition of extracellular amyloid(insoluble protein fibrils) in tissues and organs
describe the pathophysiology of amyloidosis
occurs due to abnormal folding of proteins which then aggregate and become insoluble
what are the 2 types of amyloidosis that are most common
primary/light chain(AL), secondary/systemic/inflammatory(AA)
describe the mechanism behind AL amyloidosis
production of abnormal Ig light chains from plasma cells, these enter blood stream and cause amyloid deposits
at what is AL amyloidosis usually seen in
55-60 y/o
what parts of the body are commonly affected by AL amyloidosis
heart, bowel, skin, nerves, kidneys
describe the mechanism behind AA amyloidosis
assoc. with systemic inflammation and production of acute phase protein - serum amyloid A protein(SAA)
what parts of the body does AA amyloidosis commonly affect
liver, spleen, kidneys, adrenals
what does the presentation of amyloidosis depend on
the organs and tissues involved
describe the renal presentation of amyloidosis
proteinuria(nephrotic range) +/- impaired renal function
what other presentations, other than renal, can amyloidosis present with
cardiomyopathy, neuropathy, hepato/splenomegaly, malabsorption
what renal investigations are done for amyloidosis
urinalysis + uPCR, blood tests(renal function, inflammatory markers, protein electrophoresis), renal biopsy
what is seen on renal biopsy for amyloidosis
buzz word
congo red staining shows APPLE GREEN under polarised light
describe the aims of treatment for amyloidosis
not curative, aim to reduce further deposition and preserve organ function
what is involved in the treatment of AA amyloidosis
treat the underlying condition(usually some kind of inflammation)
