Chapter 9 Renal Amyloidosis

Abstract

The kidney is one of the most frequently affected organs in several types of systemic amyloidosis. Amyloid nephropathy is diagnosed by Congo red positivity of kidney tissue and by the presence of non-branching, randomly oriented fibrils that are 8–12 nm in diameter and evident by electron microscopy. Amyloid deposits can occur in the mesangium, glomerular capillary loops, tubulo-interstitium, and/or vasculature of the kidney. Amyloid nephropathy is typically characterized by nephrotic syndrome and progression to end-stage renal disease. The rate of decline in kidney function is variable and can be slowed by treatments that reduce the production of amyloidogenic precursor proteins or new amyloid deposits.