Amyloidosis

Question 1

define

Answer 1

this is deposition of insoluble, abnormally folded proteins

Question 2

four common types

Answer 2

primary/light chain (AL)
secondary/systemic/inflammatory (AA)
dialysis and hereditary
old age

Question 3

describe AL amyloidosis

Answer 3

production of abnormal immunoglobulin light chains from plasma cells which affect the heart, bowel, skin, nerves and kidney

Question 4

age of presentation of AL

Answer 4

55-60

Question 5

describe AA amyloidosis

Answer 5

production of acute phase protein (serum amyloid A protein) associated with inflammatory conditions

Question 6

what inflammatory conditions is AA associated with?

Answer 6

RA
IBD
psoriasis
TB
osteomyelitis
bronchiectasis

Question 7

where in the body does AA affect?

Answer 7

liver
spleen
kidneys
adrenals

Question 8

presentation of amyloidosis

Answer 8

nephrotic syndrome +/- impaired function
cardiomyopathy
peripheral or autonomic neuropathy
hepatomegaly/splenomegaly and GI malabsorption

Question 9

diagnosis of amyloidosis

Answer 9

• urinalysis and bloods
• renal biopsy with Congo red staining (apple green birfringence under polarised light)
• SAP scan- scintigraphy with radiolabelled serum amyloid for extent of disease

Question 10

management of AL

Answer 10

immunosuppression e.g. steroids, chemotherapy, stem cell transplant

Question 11

management of AA

Answer 11

treat underlying condition