History of research on pathogenesis of idiopathic nephrotic syndrome

Abstract

Although nephrotic syndrome (NS) is a common kidney disease, its pathogenesis remains unclear. It is classified into idiopathic and secondary, while congenital is a third category for children. The adjective “idiopathic” is used in medicine to describe a disease or condition that has no known cause. The pathogenesis of idiopathic nephrotic syndrome (INS) remains elusive. INS is grouped into the three histological variants: minimal change NS (MCNS), focal segmental glomerulosclerosis (FSGS), and membranous nephropathy (MN). MCNS, FSGS, and MN respectively account for approximately 75-80 %, 20 %, and <3 % of INS in children, whereas each accounts for one third of INS in adults. In the past decade, advances in molecular biology have both improved our understanding of the pathogenesis of INS and created confusion. The candidate active molecules in INS, other than cytokines, include: reactive oxygen species, nuclear factor-kappa B, hemopexin, CD80 (also known as B7.1), and angiopoietin-like 4; mammalian target of rapamycin complex 1 in MCNS; cardiotrophin-like cytokine-1 and soluble urokinase-type plasminogen activator receptor in FSGS; and M-type phospholipase A2 receptor and cationic bovine serum albumin in IMN. In this review, we briefly discuss the historical background of the research on pathogenesis of INS.