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Clinical Reviews in Allergy & Immunology

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01-12-2020 | Cardiac Amyloidosis

Systemic Amyloidosis: a Contemporary Overview

Authors: Vittorio D’Aguanno, Massimo Ralli, Marco Artico, Francesca Yoshie Russo, Alfonso Scarpa, Marco Fiore, Paola Tirassa, Cinzia Severini, Marco de Vincentiis, Antonio Greco

Published in: Clinical Reviews in Allergy & Immunology | Issue 3/2020

Abstract

Amyloidosis constitutes a large spectrum of diseases characterized by an extracellular deposition of a fibrillar aggregate, generating insoluble and toxic amasses that may be deposited in tissues in bundles with an abnormal cross-β-sheet conformation, known as amyloid. Amyloid may lead to a cell damage and an impairment of organ function. Several different proteins are recognized as able to produce amyloid fibrils with a different tissue tropism related to the molecular structure. The deposition of amyloid may occur as a consequence of the presence of an abnormal protein, caused by high plasma levels of a normal protein, or as a result of the aging process along with some environmental factors. Although amyloidosis is rare, amyloid deposits play a role in several conditions as degenerative diseases. Thus, the development of antiamyloid curative treatments may be a rational approach to treat neurodegenerative conditions like Alzheimer’s disease in the future. Nowadays, novel treatment options are currently refined through controlled trials, as new drug targets and different therapeutic approaches have been identified and validated through modern advances in basic research. Fibril formation stabilizers, proteasome inhibitors, and immunotherapy revealed promising results in improving the outcomes of patients with systemic amyloidosis, and these novel algorithms will be effectively combined with current treatments based on chemotherapeutic regimens. The aim of this review is to provide an update on diagnosis and treatment for systemic amyloidosis.

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Title: Systemic Amyloidosis: a Contemporary Overview
Authors: Vittorio D’Aguanno
Massimo Ralli
Marco Artico
Francesca Yoshie Russo
Alfonso Scarpa
Marco Fiore
Paola Tirassa
Cinzia Severini
Marco de Vincentiis
Antonio Greco
Publication date: 01-12-2020
Publisher: Springer US
Keywords: Cardiac Amyloidosis
Amyloidosis
AL Amyloidosis
AA Amyloidosis
Published in: Clinical Reviews in Allergy & Immunology / Issue 3/2020
Print ISSN: 1080-0549
Electronic ISSN: 1559-0267
DOI: https://doi.org/10.1007/s12016-019-08759-4

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