Successful management of recurrent focal segmental glomerulosclerosis

Abstract

Primary focal segmental glomerulosclerosis (FSGS) recurs in up to 55% of patients after kidney transplantation. Herein we report the successful management of recurrent FSGS. A 5-year-old boy with primary FSGS received a deceased donor renal transplant. Immediate and fulminant recurrence of FSGS caused anuric graft failure that was resistant to plasmapheresis and rituximab. After exclusion of structural or immunologic damage to the kidney by repeated biopsies, the allograft was retrieved from the first recipient on day 27 and transplanted into a 52-year-old second recipient who had vascular nephropathy. Immediately after retransplantation, the allograft regained function with excellent graft function persistent now at 3 years after transplant. After 2 years on hemodialysis, the boy was listed for kidney retransplantation. To prevent FSGS recurrence, pretreatment with ofatumumab was performed. Nephrotic range proteinuria still occurred after the second transplantation, which responded, however, to daily plasma exchange in combination with ofatumumab. At 8 months after kidney retransplantation graft function is good. The clinical course supports the hypothesis of a circulating permeability factor in the pathogenesis of FSGS. Successful ofatumumab pretreatment implicates a key role of B cells. Herein we provide a description of successful management of kidney failure by FSGS, carefully avoiding waste of organs.

Keywords: disease; disease pathogenesis; domino transplantation; kidney disease; recurrent; retransplantation.

Figure 1

Electron microscopy (4000‐fold) of the allograft on day 19 after transplantation. Complete flattening of podocyte foot processes (black arrows). Minimal change lesion of incipient FSGS

Figure 2

Allograft function in the first recipient and after retransplantation into a second recipient. Renal function is depicted as diuresis (mL/24 hours) and proteinuria (protein‐creatinine ratio g/g) in the first recipient (patient 1) and after retransplantation in the second recipient (patient 2). Patient 1 received 7 sessions of plasmapheresis (black arrows) and 2 sessions of rituximab 300 mg each (black asterisk)

Figure 3

En bloc kidney retransplantation. The donor's suprarenal and infrarenal aorta were anastomosed in an end‐to‐end fashion to the recipient's external iliac artery; the donor's vena cava was anastomosed in a side‐to‐side fashion to the recipient's external iliac vein

Figure 4

Allograft function of the en bloc kidney retransplant in the 8‐year‐old recipient. Renal function is depicted as serum creatinine (mg/dL) and proteinuria (protein‐creatinine ratio g/g). The patient received 15 sessions of plasma exchange (black arrows) and ofatumumab at a dose of 1150 mg/m² (black asterisk) on day 33 posttransplant