Recurrence and Treatment after Renal Transplantation in Children with FSGS

Abstract

Focal segmental glomerulosclerosis (FSGS) is a common cause of end-stage renal disease and a common pathologic diagnosis of idiopathic nephrotic syndrome (NS), especially in steroid-resistant cases. FSGS is known to recur after kidney transplantation, frequently followed by graft loss. However, not all patients with FSGS suffer from recurrence after kidney transplantation, and genetic and secondary FSGS have a negligible risk of recurrence. Furthermore, many cases of recurrence achieve remission with the current management of recurrence (intensive plasmapheresis/immunosuppression, including rituximab), and other promising agents are being evaluated. Therefore, a pathologic diagnosis of FSGS itself should not cause postponement of allograft kidney transplantation. For patients with a high risk of recurrence who presented with classical symptoms of NS, that is, severe edema, proteinuria, and hypoalbuminemia, close monitoring of proteinuria is necessary, followed by immediate, intensive treatment for recurrence.

Figure 1

Validation of the FAST Ab panel in rFSGS and the predictive accuracy of the subsets of this panel. ROC analysis for three fitted logistic regression models. The outcome was recurrence versus nonrecurrence of FSGS, and the independent predictors were the log-transformed relative fluorescent signal values of seven Abs: CD40, PTPRO, FAS, CGB5, SNRPB2, APOL2, and P2RY11. The three logistic regression models fitted are shown. Model 1 used the FAST (FSGS antibody serological test) panel with all seven Abs, resulting in an AUC = 0.9. Model 2 used three Abs (CD40, PTPRO, and CGB5), and its ROC curve had an AUC of 0.82. Model 3 used only CD40 Ab data for the ROC analysis, resulting in an AUC of 0.77. Reproduced with permission from The American Association for the Advancement of Science © 2014 (AAAS), Delville et al. [45].

Figure 2

Scatter plot of VASP phosphorylation levels by patient group. Phosphorylation for each individual sample was assigned a densitometry value relative to the control (normal) plasma sample from the same gel, which was normalized to 1. Reproduced with permission from Wiley © 2012 Pathological Society of Great Britain and Ireland, Harris et al. [46].

Figure 3

Approach for patients with SRNS-FSGS, authors' suggestion.