Amyloidosis Flashcards
Define amyloidosis
A group of conditions caused by the build-up of amyloid (abnormal protein) in tissues and organs
Name risk factors for amyloidosis
Age Male Chronic/inflammatory diseases FH Kidney dialysis (inefficient at removing large proteins eg amyloid) African heritage
What is the aetiology of amyloidosis
During protein synthesis, misfolded antibodies fail to get hydrolysed (destroyed) because of their robustness, and therefore aggregate to form oligomers, which further aggregate to form amyloid fibrils
These fibrils get deposited into organs
Describe the epidemiology of amyloidosis
RA and IBD common causes
Common between 60-70y
How does amyloidosis present?
Fatigue, weakness SOB Numbness in hands/feet (carpal tunnel) Wt loss Dysphagia Diarrhoea Enlarged tongue
What signs of amyloidosis can be found on examination?
Arrythmias Pedal oedema (HF) Orthostatic hypotension
What investigations might you do for amyloidosis?
Tissue biopsy and search for amyloid
Protein electropheresis
Bloods, echo, CT/MRI of affected organs
What is the management plan for amyloidosis?
No cure
Chemo to destroy abnormal bone marrow cells
+steroids? to increase effect of chemo
Where is amyloid made?
Bone marrow
Which parts of the body are most commonly affected by amyloidosis?
Heart, kidneys, liver, spleen, NS, digestive tract
Primary amyloidosis is also known as what?
AL amyloidosis
Secondary amyloidosis is also known as what?
AA amyloidosis
