| Abstract|| |
Focal segmental glomerulosclerosis (FSGS) may recur following transplantation. Approximately half of the patients with recurrent FSGS lose their grafts. We report a case of a 54-year-old woman with focal segmental glomerulosclerosis (FSGS). She underwent live-related donor kidney transplantation from her 21-year-old son and immunosuppression was maintained with tacrolimus, mycophenolate mofetil and steroids. Eight months after transplantation, the patient presented with increasing lower limbs edema and heavy proteinuria. Allograft biopsy contained 20 glomeruli of which four were totally sclerosed, seven were segmentally sclerotic, and the rest were non-sclerotic, relatively enlarged glomeruli compatible with recurrent FSGS in the graft. Plasmapheresis over a two week period with simultaneous oral cyclophosphamide resulted in a partial response of proteinuria from 12 to 2.2 g/24hrs over 5 weeks.
Keywords: Focal segmental glomerulosclerosis, renal transplantation
|How to cite this article:|
Akash N. Recurrence of Focal Segmental Glomerulosclerosis after Renal Transplantation: A Case Report. Saudi J Kidney Dis Transpl 2007;18:91-4
|How to cite this URL:|
Akash N. Recurrence of Focal Segmental Glomerulosclerosis after Renal Transplantation: A Case Report. Saudi J Kidney Dis Transpl [serial online] 2007 [cited 2020 Jul 9];18:91-4. Available from: http://www.sjkdt.org/text.asp?2007/18/1/91/31853
| Introduction|| |
Primary Focal segmental glomeruloscloresis (FSGS) is a clinicopathological syndrome of moderate to severe proteinuria, early onset of hypertension, steroid resistance, progressive azotemia, and typical glomerular lesions.,,,
FSGS may recur after renal transplantation with heavy proteinuria, hypertension and allograft dysfunction, ,, with the estimated frequency of recurrence of FSGS varying from 20% to 40%, , with graft loss in 40% to 50% of those affected ,. Recurrence may be encountered within hours after transplantation, , and usually occurs within the first year in 80% of cases. 
| Case Report|| |
A 54-year-old woman with focal segmental glomerulosclerosis (FSGS) was started on regular hemodialysis for her endstage renal disease three years after the diagnosis of the disease was established.
The patient was referred for work-up at our center and possible kidney transplanttation. She had negligible urine output and insignificant proteinuria at that stage.
In October 2002, she underwent liverelated donor kidney transplantation; the donor was her 21-year-old son. She was started on a triple immunosuppressive regimen including tacrolimus, mycophenlate mofetil and steroids. She had a smooth post-operative course and was discharged from hospital one week later with normal kidney function and urinalysis. She was then followed up regularly at the nephrology clinic and all her laboratory data were within normal range.
In June 2003, the patient presented with increasing lower limbs edema of 2 weeks duration with significant proteinuria of 12gm/24hrs along with hypoalbuminemia and normal kidney function. She underwent an allograft biopsy that contained 20 glomeruli of which four were totally sclerosed, seven were segmentally sclerotic, and the rest were non-sclerotic relatively enlarged glomeruli compatible with recurrent FSGS in the graft, [Figure - 1].
The patient was subjected to a course of nine sessions of plasmapheresis (1 plasma volume/exchange) over a two week period with simultaneous oral cyclophosphamide that substituted mycophenolate mofetil for three months. Subsequently, she had a substantial decrease in her proteinuria from 12 to 2.2 g/24hrs over 5 weeks.
| Discussion|| |
The pathogenesis of FSGS is largely unknown, although clinical and experimental observations suggest that a circulating plasma factor is responsible for protein leaks in patients with recurrent FSGS after transplantation.  The beneficial effect of plasmapheresis and immunoadsorption with protein A in inducing remission of proteinuria provides additional support for a circulating FSGS permeability factor (FSPF). 
The FSPF identified by Savin and colleagues is a small glycoprotein present in plasma at very low concentrations and may be protein-bound in some conditions. , In certain cases, inhibitory substances that are present in normal serum may play a role in protecting glomeruli from proteinuria. 
Several centers have reported the success of plasmapheresis and immunoabsorption with protein A in inducing remission in most patients treated within two weeks of relapse. , ,,, Remission may result from the removal of the plasma permeability factor. However, many patients would relapse when plasmapheresis is discontinued.
Treatment with cyclosporine may decrease proteinuria in recurrent FSGS and in the primary form. Nevertheless, the inclusion of cyclosporine in the immunosupression protocol does not usually prevent recurrence. 
Cyclophosphamide with plasmapheresis has induced remission in some patients with post-transplant FSGS.  In our patient, there was a partial but significant decrease of proteinuria.
In conclusion, FSGS can recur after renal transplantation and the prognosis is poor in untreated patients. A circulating plasma factor has been implicated in the pathogenesis of recurrent FSGS and treatment with plasma pheresis has proven effective in decreasing proteinuria in some patients.
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