Abstract
Amyloidosis is an uncommon disease that is characterized by abnormal extracellular deposition of misfolded protein fibrils leading to organ dysfunction. The deposited proteins display common chemical and histologic properties but can vary dramatically in their origin. Kidney disease is a common manifestation in patients with systemic amyloidosis with a number of amyloidogenic proteins discovered in kidney biopsy specimens. The emergence of mass spectrometry-based proteomics has added to the diagnostic accuracy and overall understanding of amyloidosis. This in-depth review discusses the general histopathologic features of renal amyloidosis and includes an in-depth discussion of specific forms of amyloid affecting the kidney.