Amyloidosis

Question 1

Pathophysiology

Answer 1

Build up of too much or aberrant proteins elsewhere in the body.

They can’t be broken down by proteases because of their difficult structure or because there is too much of the protein being produced.

Question 2

What are the two classifications of amyloidosis?

Answer 2

Local and systemic

Question 3

Name two types of systemic amyloidosis and explain these

Answer 3

AL - amyloid light chain (from bone marrow, e.g. from myeloma)
AA - amyloid A protein (normal protein released in acute phase reactions, therefore when inflammation prolonged e.g. in cancer, RA, released)

Question 4

Explain the presentation and what happens when amyloid proteins build up in the kidneys

Answer 4

Deposited in podocytes lining glomerulus. This causes leakage of albumin into urine. End up with nephrotic syndrome.

Question 5

Key features of nephrotic syndrome and explain

Answer 5

Hypoalbuminaemia
Albuminuria
Hyperlipidemia (because albumin is needed to stop lipid synthesis)
Oedema

Question 6

Explain the presentation and what happens when amyloid proteins build up in the heart

Answer 6

Restrictive cardiomyopathy -> CHF
Arrhythmias

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