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Amyloidosis
From WikEM
Contents
Background
- Accumulation of proteins (amyloid fibrils) in tissues
- Heart and kidneys most commonly affected organs
- Causes:
- Primary amyloidosis, hereditary amyloidosis
- Secondary (due to systemic inflammatory condition)
- ESRD-associated (caused by β2-microglobulin amyloids, not filtered out by dialysis membranes))
- Senile amyloidosis (causes cardiac amyloid)
Clinical Features
- Nephrotic syndrome with kidney involvement
- Restrictive cardiomyopathy
- Sensory and autonomic neuropathies (no CNS involvement)
- Sensory neuropathy: symmetrical pattern, progresses distal to proximal
- Autonomic neuropathy: orthostatic hypotension or nonspecific GI symptoms
- Hepatomegaly, elevated serum AST and AlkPhos
- Diabetes secondary to pancreatic infiltration
- Macroglossia
Differential Diagnosis
Evaluation
- Evaluate for other causes of symptoms
- Diagnosis via fat pad biopsy: apple-green birefringence with Congo red stain
