Amyloidosis is a disease caused by deposition of amyloid protein in various tissues and organs, and is also an important cause of chronic renal failure, usually occurring within 3 years of the development of nephrotic syndrome. To date, 13 types of amyloid proteins have been identified in humans, regardless of systemic or localized amyloidosis. Amyloid proteins derived from serum amyloid A (AA), immunoglobulin light chains (AL), transthyretin, and β2-microglobulin are constituents in systemic amyloidosis. Despite the close histological resemblance of each amyloid deposit, the pathogenesis appears to differ according to individual amyloidosis.