Optimal management of primary focal segmental glomerulosclerosis in adults

Abstract

Séverine Beaudreuil,1,2 Hans Kristian Lorenzo,1,2 Michele Elias,1 Erika Nnang Obada,1 Bernard Charpentier,1,2 Antoine Durrbach1,2 1Department of Nephrology Dialysis Transplantation, Paris-Sud University Hospital, Le Kremlin Bicêtre, 2INSERM Unit 1197, Paris-Sud University Hospital, Villejuif, France Abstract: Focal segmental glomerulosclerosis (FSGS) is a frequent glomerular kidney disease that is revealed by proteinuria or even nephrotic syndrome. A diagnosis can be established from a kidney biopsy that shows focal and segmental glomerulosclerosis. This histopathological lesion may be caused by a primary podocyte injury (idiopathic FSGS) but is also associated with other pathologies (secondary FSGS). The first-line treatment for idiopathic FSGS with nephrotic syndrome is a prolonged course of corticosteroids. However, steroid resistance or steroid dependence is frequent, and despite intensified immunosuppressive treatment, FSGS can lead to end-stage renal failure. In addition, in some cases, FSGS can recur on a graft after kidney transplantation: an unidentified circulating factor may be implicated. Understanding of its physiopathology is unclear, and it remains an important challenge for the scientific community to identify a specific diagnostic biomarker and to develop specific therapeutics. This study reviews the treatment of primary FSGS and the recurrence of FSGS after kidney transplantation in adults. Keywords: glomerulosclerosis, kidney transplantation, circulating factor, treatment 

Keywords

• glomerulosclerosis
• kidney transplantation
• circulating factor
• treatment