Amyloidosis
Background
- accumulation of proteins (amyloid fibrils) in tissues
- heart and kidneys most commonly affected organs
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 sx
- Hepatomegaly, elevated serum AST and AlkPhos
- Diabetes secondary to pancreatic infiltration
- Macroglossia
Differential Diagnosis
Evaluation
- diagnosis via fat pad biopsy: apple-green birefringence with Congo red stain