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Amyloidosis: Immunopathogenesis and diagnosis 

Amyloidosis: Immunopathogenesis and diagnosis
Chapter:
Amyloidosis: Immunopathogenesis and diagnosis
Author(s):

H.J. Lachmann

and P.N. Hawkins

DOI:
10.1093/med/9780199579655.003.0102
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date: 09 August 2020

Amyloidosis is a disorder of protein folding in which normally soluble plasma proteins are deposited in the extracellular space in an abnormal insoluble fibrillar form. Accumulation of these fibrils causes progressive disruption of the structure and function of any body tissue or organ. Without treatment systemic disease is usually fatal, but measures that reduce the supply of amyloid fibril precursor proteins can frequently lead to regression of amyloid deposits, prevention of organ failure and improved survival. However, as the regression of amyloid is relatively slow, early diagnosis greatly improves the prognosis. Amyloid is remarkably diverse and can be hereditary or acquired, localized or systemic, and lethal or merely an incidental finding. It is rare but not especially so, with systemic amyloidosis being responsible for about one in 1500 deaths in the UK.

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