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Currents concepts on the immunopathology of amyloidosis
Clinical Reviews in Allergy and Immunology, 07/28/09
Bhat A et al. – Review article focuses on the pathogenesis and clinical features of amyloidosis, that is defined as the extracellular accumulation of insoluble low molecular weight protein fibrils at systemic or organ-specific level. Clinical presentation varies by the number and nature of affected organs, as in some cases only one organ is affected, while in others there is extensive multisystem involvement:
- Kidney (80%): Nephrotic syndrome, hypoabluminemia, edema, and/or anasarca, Azotemia without proteinuria (rare)
- Heart (40%): Diastolic heart failure, Increased interventricular septal thickness, low voltage on electrocardiogram with a pseudo infarct pattern
- Nervous system: Peripheral nervous system (18%), carpal tunnel syndrome (25%), orthostatic hypotension (16%), autonomic dysfunction with gastrointestinal symptoms
- Oral (10%): Macroglossia
- Digestive (34%): Hepatosplenomegaly, hyposplenism without significant splenomegaly
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