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Abstract
Background: Amyloid A (AA) amyloidosis is a serious complication of a wide range of chronic inflammatory, infectious and neoplastic diseases. A longstanding overproduction of the liver-synthesised cytokine-induced acute phase serum amyloid A (SAA) protein is a key event in the pathogenetic cascade leading to the deposition of AA amyloid in tissues and organs. Objective: The aim of the study was to critically review treatment strategies in AA amyloidosis. Methods: A systematic literature review was conducted based on PubMed (January 1980 – April 2008) and selected conference abstracts. Results/conclusions: The current strategy for the treatment of AA amyloidosis is firmly based on the knowledge of the underlying pathogenetic mechanism and aims at reducing the amyloid precursor (SAA) load by intensive anti-inflammatory/immunosuppressive therapy and, in selected instances, anticytokine (TNF-α, IL-1β or IL-6 blockade) therapy, or, when applicable, the eradication of an existing infectious focus (surgery, antimicrobial drugs). Emerging strategies focus on the dissolution of the amyloid deposits using small molecules that either interact with the glycosaminoglycans or the fibril component of the deposits, or deplete amyloid P component.