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Protein Aggregation and Fibrillogenesis in Cerebral and Systemic Amyloid Disease pp 541–564Cite as

Systemic AA Amyloidosis

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Part of the book series: Subcellular Biochemistry ((SCBI,volume 65))

Abstract

Systemic AA amyloidosis is a rare complication of chronic inflammatory disorders. The amyloid fibrils are derived from serum amyloid A protein, an acute phase protein synthesized in the liver. Clinical presentation is most commonly due to the consequences of renal involvement, with proteinuria and progressive renal decline. Progression to end stage renal failure is common. Management is currently centred on reducing the supply of the precursor protein by treating the underlying inflammatory condition, whilst supporting the affected organs. Monitoring of the serum amyloid A protein is vital to assess whether there is adequate suppression of the underlying disease. The level of serum amyloid A protein is a powerful predictor of both patient survival and renal outcome. In patients with adequate suppression of the serum amyloid A protein amyloid deposits can be seen to regress and renal function can be stabilised and even improve.

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Authors and Affiliations

  1. National Amyloidosis Centre and Centre for Nephrology, Division of Medicine, UCL Medical School, Rowland Hill Street, NW3 2PF, London, UK

    Jennifer H. Pinney

  2. National Amyloidosis Centre and Centre for Nephrology, Division of Medicine, UCL Medical School, Royal Free Campus, NW3 2QG, London, UK

    Jennifer H. Pinney & Helen J. Lachmann

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  1. Jennifer H. Pinney
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  2. Helen J. Lachmann
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Correspondence to Jennifer H. Pinney .

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  1. Inst. of Zoology, University of Mainz, Becherweg 6, Mainz, 55099, Germany

    J. Robin Harris

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© 2012 Springer Science+Business Media Dordrecht

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Pinney, J., Lachmann, H. (2012). Systemic AA Amyloidosis. In: Harris, J. (eds) Protein Aggregation and Fibrillogenesis in Cerebral and Systemic Amyloid Disease. Subcellular Biochemistry, vol 65. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-5416-4_20

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