Update on the treatment of focal segmental glomerulosclerosis in renal transplantation

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.

What Can We Do When All Collapses? Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report

“…Treatment of recurrent post-transplantation FSGS has not reached a consensus but, in the majority of centers, the first therapeutic approach is the same as the one used in this patient, with corticosteroid pulses and plasmapheresis [1,9]. Other therapeutic strategies are also available, such as use of rituximab, but there is still no consensus on the timing, dose, or duration of therapy [10].…”

Extracorporeal Therapies in the Treatment of Focal Segmental Glomerulosclerosis

“…FSGS is a histological pattern of glomerular lesion defined by both clinical and pathologic findings. FSGS is either classified as primary (idiopathic) or as secondary due to a wide array of causes, such as illicit drug use, HIV, or other viral infections [6]. Primary FSGS (pFSGS) is currently the most common cause of ESRD in the United States (US) and has shown to occur most commonly in children and young adults.…”

Histopathological findings in transplanted kidneys

“…The reported risk of recurrence of focal segmental glomerulosclerosis (FSGS) in the first graft ranges from 30 to 60%, whereas the rate approaches 100% in subsequent grafts [147]. Clinical features of FSGS recurrence include the early and acute onset of massive proteinuria (hours to days after transplantation).…”

“…However, the significance of suPAR is still controversial [152]. In addition, novel candidates such as CLC-1, anti-CD40 Ab, and vasodilator-stimulated phosphoprotein are proposed [153]. The pathological significance of variant transition remains unknown.…”