Amyloidosis Flashcards
What is it?
amyloidosis is a term which describes the extracellular deposition of an insoluble fibrillar protein termed amyloid
i.e. the disease of the tissue damage caused by deposition of abnormal protein formations (beta sheets) in tissue
Diagnosis
Biopsy - Congo red staining: apple-green birefringence under polarised light
serum amyloid precursor (SAP) scan
biopsy of rectal tissue
Causes
AL vs AA
AL amyloidosis:
- Myeloma where plasma cells produce too many light chains
AA amyloidosis: serum amyloid A is released when there is inflammation so when there is chronic inflammation too much gets released
- Rheumatoid Arthritis
- Inflammatory bowel disease
- Cancer
- Familial mediterranean fever
Manifestations
Restrictive cardiomyopathy
Arrhythmia
Nephortic syndrome
Neuropathy: autonomic and peripheral (central nervous s
Alzheimer’s disease is a form of localised amyloidosis
Diabetes
Large tongue
Essentially depends where it affects
Management
Organ transplant