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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2007  |  Volume : 18  |  Issue : 1  |  Page : 91-94
Recurrence of Focal Segmental Glomerulosclerosis after Renal Transplantation: A Case Report


Renal Unit, Department of Medicine, King Hussein Medical Center, Amman, Jordan

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   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 2019 Nov 22];18:91-4. Available from: http://www.sjkdt.org/text.asp?2007/18/1/91/31853

   Introduction Top


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.[1],[2],[3],[4]

FSGS may recur after renal transplantation with heavy proteinuria, hypertension and allograft dysfunction, [5],[6],[7] with the estimated frequency of recurrence of FSGS varying from 20% to 40%, [8],[9] with graft loss in 40% to 50% of those affected [10],[11]. Recurrence may be encountered within hours after transplantation, [9],[12] and usually occurs within the first year in 80% of cases. [9]


   Case Report Top


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 Top


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. [13] 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). [14]

The FSPF identified by Savin and colleagues[14] is a small glycoprotein present in plasma at very low concentrations and may be protein-bound in some conditions. [15],[16] In certain cases, inhibitory substances that are present in normal serum may play a role in protecting glomeruli from proteinuria. [17]

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. [9], [15],[16],[17],[18] 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.[19] Nevertheless, the inclusion of cyclosporine in the immunosupression protocol does not usually prevent recurrence. [20]

Cyclophosphamide with plasmapheresis has induced remission in some patients with post-transplant FSGS. [21] 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.

 
   References Top

1.Schwartz MM, Korbet SM. Primary focal segmental glomerulosclerosis: pathology, histological variants and patho­genesis. Am J Kidney Dis 1993;22:874-83.  Back to cited text no. 1  [PUBMED]  
2.Rich AR. A hitherto undescribed volnerability of the juxtamedullary glomeruli in lipoid nephrosis. Bull Johns Hopkins Hosp 1957;100:173-86.  Back to cited text no. 2  [PUBMED]  
3.White RH, Glascow EF,Mills RJ. Clinicopathological study of nephritic syndrome in childhood. Lancet 1970;1:1353-9.  Back to cited text no. 3    
4.Korbet SM, Schwartz MM, Lewis EJ. The prognosis of focal segmental glomerulosclerosis of adulthood. Medicine 1986;65:304-11.  Back to cited text no. 4  [PUBMED]  
5.Kim EM, Striegel J, Kim Y, Matas AJ, Najarian JS, Mauer SM. Recurrence of steroid-resistance nephritic syndrome in kidney transplants is associated with increased acute renal failure and acute rejection. Kidney Int 1994;45:1440-5.  Back to cited text no. 5  [PUBMED]  
6.Baum MA Stablein DM, Panzarino VM, et al. Loss of living donor renal allograft survival advantage in children with focal segmental glomerulo­sclerosis.Kidney Int 2001;59:328-33.  Back to cited text no. 6    
7.Briggs JD, Jones E. Recurrence of glomerulonephritis following renal transplantation.Nephrol Dial Transplant 1999;14:564-5.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Neumayer HH, Kienbaum M, Graf S, Schreiber M, Mann JFB, Luft FC. Prevalence and long-term outcome of glomerulonephritis in renal allografts. Am J Kidney Dis 1993;22:320-5.  Back to cited text no. 8    
9.Dantal J, Baatard R, Hourmant M, Cantarovitch D, Buzelin F, Soulillou JP. Recurrent nephrotic syndrome following renal transplantation in patients with focal glomerulosclerosis. Transplantation 1991;52:827-31.  Back to cited text no. 9    
10.Tejani A, Stablein DH.Recurrence of focal segmental glomerulosclerosis post-transplantation. J Am Soc Nephrol 1992;2(suppl 12):s256-63.  Back to cited text no. 10    
11.Artero M, Biava C, Amend W, Tomlanovich S, Vincenti F. Recurent focal glomerulosclerosis: natural history and response to therapy. Am J Med 1992,92:375-83.  Back to cited text no. 11  [PUBMED]  [FULLTEXT]
12.Holgado R, Del castillo D, Mazuecos A, et al. Long term out come of focal segmental glomerulosclerosis after renal transplantation.Transplant Proc 1999;31:2304-5.  Back to cited text no. 12  [PUBMED]  [FULLTEXT]
13.Andresdettir MB, Ajubi N, Croockewit S, Assmann KJ, Hibrands LB, Wetzels JF. Recurrent focal glomerulosclerosis: natural course and treatment with plasma exchange. Nephrol Dial Transplant 1999;14:2650-56.  Back to cited text no. 13    
14.Gohh RY, Yango AF, Morrissey PE, et al. Preemptive plasmapheresis and recurrence of FSGS in high risk renal transplant recipients. Am J Transplant 2005;5:2907-12.  Back to cited text no. 14  [PUBMED]  [FULLTEXT]
15.Artero ML, Sharma R, Savin VJ, Vincenti F. Plasmapheresis reduces proteinuria and serum capacity to injure glomeruli in patients with recurrent focal glomerulosclerosis. Am J Kidney Dis 1994;23:574-81.  Back to cited text no. 15  [PUBMED]  
16.Savin VJ, Sharma R, Sharma M, et al. Circulating factor associated with increased glomerular permeability to albumin in recurrent focal segmental glomerulosclerosis. N. Engl J Med 1996;334:878-83.  Back to cited text no. 16    
17.Sharma M, Sharma R, Mecarthy ET, Savin VJ. The focal segmental glomerulosclerosis permeability factor: Biochemical characteristics and biological effects. Exp Biol Med 2004;229:85-98.  Back to cited text no. 17    
18.Sharma R, Sharma M, Mc Carthy ET, GE XL, Savin VJ. Components of normal serum block the focal segmental glomerulosclerosis factor activity in vitro.Kidney Int 2000;58(5),1973-9.  Back to cited text no. 18    
19.Haas M, Godfrin Y, Oberbauer R, et al. Plasma immunadsorptiontreatment in patients with primary focal and segmental glomerulosclerosis.Nephrol Dial Transplant 1998;13:2013-16.  Back to cited text no. 19  [PUBMED]  [FULLTEXT]
20.Kershaw DB, Sedman EB, Kelsch RC, Bunchman .Recurrent focal segmental glomerulosclerosis in pediatric renal transplant recipients: successful treatment with oral cyclophosphamide. Clin Transplant 1994;8(6):546-9.  Back to cited text no. 20    
21.Banfi G, Colturi C, Montagnino G, Ponticelli C. The recurrence of focal segmental glomerulosclerosis in kidney transplant patients treated with cyclosporine. Transplantation 1990; 50:594-6.  Back to cited text no. 21  [PUBMED]  

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Correspondence Address:
Nabeel Akash
P.O.Box 1362, Amman 11953
Jordan
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PMID: 17237899

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