Review
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Transplant. Mar 24, 2016; 6(1): 54-68
Published online Mar 24, 2016. doi: 10.5500/wjt.v6.i1.54
Update on the treatment of focal segmental glomerulosclerosis in renal transplantation
Maria Messina, Ester Gallo, Alberto Mella, Fabiola Pagani, Luigi Biancone
Maria Messina, Ester Gallo, Alberto Mella, Fabiola Pagani, Luigi Biancone, Renal Transplantation Center “A. Vercellone”, Division of Nephrology Dialysis and Transplantation, Department of Medical Sciences, Città della Salute e della Scienza Hospital and University of Turin, 10126 Turin, Italy
Author contributions: Messina M, Gallo E, Mella A, Pagani F and Biancone L equally contributed to conception and design of the study, literature review, and analysis, drafting, critical revision and editing of the manuscript; all authors approved the final version.
Conflict-of-interest statement: None of the authors has any potential conflicts of interest related to this study.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Luigi Biancone, MD, PhD, Renal Transplantation Center “A. Vercellone”, Division of Nephrology Dialysis and Transplantation, Department of Medical Sciences, Città della Salute e della Scienza Hospital and University of Turin, Corso Bramante 88, 10126 Turin, Italy. luigi.biancone@unito.it
Telephone: +39-011-6336797 Fax: +39-011-6334990
Received: November 15, 2015
Peer-review started: November 16, 2015
First decision: December 7, 2015
Revised: December 22, 2015
Accepted: January 21, 2016
Article in press: January 22, 2016
Published online: March 24, 2016
Abstract

Focal segmental glomerulosclerosis (FSGS) represents one of the most severe glomerular diseases, with frequent progression to end-stage renal disease and a high rate of recurrence in renal allografts (30%-50%). Recurrent FSGS portends a negative outcome, with the hazard ratio of graft failure being two-fold higher then that of other glomerulonephritis. Two patterns of clinical presentations are observed: Early recurrence, which is characterized by massive proteinuria within hours to days after implantation of the renal graft, and late recurrence, which occurs several months or years after the transplantation. Many clinical conditions have been recognized as risk factors for recurrence, including younger age, rapid progression of the disease to end-stage renal disease on native kidneys, and loss of previous renal allografts due to recurrence. However, much less is known about the incidence and risk factors of the so-called “de novo” type of FSGS, for which sufferers are transplanted patients without disease on native kidneys; but, rapid development of allograft failure is frequently observed. Management of both forms is challenging, and none of the approaches proposed to date have been demonstrated as consistently beneficial or effective. In the present review we report an update on the available therapeutic strategies for FSGS in renal transplantation within the context of a critical overview of the current literature.

Keywords: Focal segmental glomerulosclerosis, Kidney transplantation, Permeability factors, Plasma exchange, Rituximab

Core tip: Focal segmental glomerulosclerosis (FSGS) presents as a histological pattern of kidney damage with different, multifactorial, and often undefined pathogenesis. Primary FGSS represents one of the most severe glomerular diseases, with frequent progression to end-stage renal failure and a high rate of recurrence in renal allografts. FSGS recurrence also portends a negative outcome. Despite the proposal of multiple therapeutic approaches, none has emerged as the resolutive option. This review provides an update on the currently available therapeutic strategies for FSGS in renal transplantation, along with a critical overview of the related literature.