Systemic AA amyloidosis

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.