Systemic Amyloidosis

Abstract

Systemic amyloidosis is characterized by the extracellular deposition of abnormal fibrillar protein. There are different types of amyloid, defined by their respective fibril precursor proteins. The pattern of organ involvement and dysfunction varies substantially, not only between different amyloid types, but also within each type. The relative rarity, along with varied clinical presentations and requirement for a correctly stained histological specimen, make diagnosis of amyloidosis challenging. The possibility of systemic amyloidosis is often overlooked, leading to a substantial delay in diagnosis and a high index of suspicion is thus required. Treatment revolves around eliminating or reducing the supply of precursor protein so that amyloidogenesis is switched off and regression of existing deposits can occur. Meticulous supportive care of organ function during treatment is imperative. © 2012 Blackwell Publishing Ltd.