[Amyloidoses].

Abstract

Amyloidoses are rare diseases, defined by the accumulation of extracellular deposits with ultrastructural fibrillary organization, and molecular beta-pleated conformation. Amyloidoses are defined by the type of protein aggregates, the most common being immunoglobulin light-chains amyloidosis (AL). The treatment of AL amyloidosis has recently been improved by serum immunoglobulin light chains assay for close treatment monitoring; prognostic markers of cardiac damage such as BNP, NT-proBNP and troponin; and the emergence of new anti plasma cell drugs. AA amyloidosis should be screened by a search of proteinuria in every patient with chronic inflammatory disease. To diagnose rare hereditary amyloidoses requires to gather clinical, pathological and genetic data. Recent therapeutic advances showed efficacy of a molecule stabilizing transthyretin in early forms of amyloid neuropathy due to transthyretin mutations.

• Authors: Sophie Georgin-Lavialle, Katia Stankovic Stojanovic, David Buob, Gilles Grateau
• Journal: La Revue du praticien (Rev Prat) Vol. 64 Issue 8 Pg. 1050-3 (Oct 2014) ISSN: 0035-2640 [Print] France
• Vernacular Title: Amyloses.
• PMID: 25510121 (Publication Type: English Abstract, Journal Article)

Topics

• Amyloidosis (diagnosis, etiology)
• Humans