Recurrent nephrotic syndrome with three successive renal allografts

A girl with idiopathic steroid-resistant nephrotic syndrome was studied at onset of the disease at 6 years of age and during recurrences of the nephrotic syndrome after successive transplantations of three renal allografts. Focal glomerulosclerosis was demonstrated shortly after onset of the original disease with progression to renal insufficiency over 14 months. Recurrence of the nephrotic syndrome was documented 4 weeks following transplantation of the first renal allograft and a biopsy demonstrated focal segmental glomerulosclerosis without evidence of rejection. By 13 months, recurrent disease had caused renal failure requiring dialysis. After transplantation of the next long-functioning graft, the nephrotic syndrome rapidly recurred with urinary protein excretion of 8 g/day and normal glomerular filtration rate at 8 weeks. After the 5th month, progressive renal insufficiency developed and dialysis was resumed at 10 months after transplantation. The nephrectomy specimen showed advanced focal segmental glomerulosclerosis and chronic rejection. Early after the last renal allograft, she had mild persistent proteinuria and then developed nephrotic syndrome at 10 months which was attributed to chronic rejection. The graft was lost to delayed accelerated rejection at 17 months. The rapid recurrence of nephrotic syndrome after transplantation of each of the first two long-functioning allografts to this patient is most consistent with recurrence of original disease. However, observations in a subsequent graft demonstrated that rapid recurrence of the original disease is not inevitable.