Amyloidosis: Difference between revisions
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*Restrictive [[cardiomyopathy]] | *Restrictive [[cardiomyopathy]] | ||
*Sensory and autonomic neuropathies (no CNS involvement) | *Sensory and autonomic neuropathies (no CNS involvement) | ||
**Sensory neuropathy: symmetrical pattern, progresses distal to proximal | **[[numbness|Sensory neuropathy]]: symmetrical pattern, progresses distal to proximal | ||
**Autonomic neuropathy: orthostatic hypotension or nonspecific GI symptoms | **Autonomic neuropathy: orthostatic [[hypotension]] or nonspecific GI symptoms | ||
*Hepatomegaly, elevated serum AST and AlkPhos | *[[Hepatomegaly]], elevated serum AST and AlkPhos | ||
*[[Diabetes]] secondary to pancreatic infiltration | *[[Diabetes]] secondary to pancreatic infiltration | ||
*[[Macroglossia]] | *[[Macroglossia]] | ||
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==Management== | ==Management== | ||
*Varies depending on underlying etiology and organ systems involved | |||
==Disposition== | ==Disposition== | ||
Revision as of 22:09, 1 October 2019
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
Management
- Varies depending on underlying etiology and organ systems involved