title: [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.forename: Sophie
authors.initials: S
authors.lastname: Georgin-Lavialle
authors.forename: Katia Stankovic
authors.initials: KS
authors.lastname: Stojanovic
authors.forename: David
authors.initials: D
authors.lastname: Buob
authors.forename: Gilles
authors.initials: G
authors.lastname: Grateau
mesh_terms: D000686:Amyloidosis / Q000175:diagnosis / Q000209:etiology; D006801:Humans