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Recurrent nephrotic syndrome after transplantation: Early treatment with plasmaphaeresis and cyclophosphamide

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Abstract

Steroid-resistant nephrotic syndrome (NS) with focal glomerulosclerosis (FGS) and its recurrence after transplantation are mainly seen in children. The recurrence rate approximates 30% and the graft loss is about half this. Several therapeutic regimens have been proposed, giving conflicting results. In an attempt to remove a putative circulating factor and inhibit its production by lymphocytes, three patients with biopsy-proven FGS in the native kidney were included in a prospective uncontrolled trial using early plasmaphaeresis followed by substitutive immunoglobulins in association with methylprednisolone pulses and cyclophosphamide instead of azathioprine over a 2-month period. The patients were girls, aged 6.5, 13.3 and 15.8 years, who received a cadaveric transplant; concomitant immunosuppression included prednisone and cyclosporine A. All three patients exhibited early recurrence of the NS and were treated 5–10 days after the onset of proteinuria. Rapid and sustained remission was achieved in all patients within 12–24 days on therapy. One patient experienced a late acute but steroid-sensitive rejection episode; another suffered from septic ankle arthritis as a complication of reinforced immunosuppression. The latter girl had a second late recurrence of proteinuria that was controlled within 7 weeks. With a 18-to 27-month follow-up, all three patients have normal renal function, normal blood pressure and no proteinuria. We conclude that intensive therapy using plasmaphaeresis, steroid pulses and cyclophosphamide over a 2-month period can induce complete remission in children with early recurrence of NS after transplantation.

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References

  1. Streigel JE, Sibley RK, Fryd DS, Mauer SM (1986) Recurrence of focal segmental sclerosis in children following renal transplantion. Kidney Int 30: S44-S50

    Google Scholar 

  2. Senggutuvan P, Cameron JS, Hartley RB, Rigden S, Chantler C, Haycock G, Williams DG, Ogg C, Koffman G (1990) Recurrence of focal segmental glomerulosclerosis in transplanted kidneys: analysis of incidence and risk factors in 59 allografts Pediatr Nephrol 4: 21–28

    PubMed  Google Scholar 

  3. Rizzoni G, Ehrich JHH, Brunner FP, Geerlings W, Fassbinder W, Landais P, Mallick N, Margreiter R, Raine AEG, Selwood NH, Tufveson G (1991) Combined report on regular dialysis and transplantation of children in Europe, 1990. Nephrol Dial Transplant 6: 31–42

    Google Scholar 

  4. Cameron JS (1991) Recurrent primary disease and de novo nephritis following renal transplantation. Pediatr Nephrol 5: 412–421

    PubMed  Google Scholar 

  5. Stephanian E, Matas AJ, Mauer SM, Chavers B, Nevins T, Kashtan C, Sutherland DER, Gores P, Najarian JS (1992) Recurrence of disease in patients retransplanted for focal segmental glomerulosclerosis. Transplantation 53: 755–757

    PubMed  Google Scholar 

  6. Artero M, Biava C, Amend W, Tomlanovich S, Vincenti F (1992) Recurrent focal glomerulosclerosis: natural history and response to therapy. Am J Med 92: 375–383

    PubMed  Google Scholar 

  7. Dantal J, Testa A, Bigot E, Soulillou JP (1990) Disappearance of proteinuria after immunoadsorption in a patient with focal glomerulosclerosis. Lancet 336: 190

    Google Scholar 

  8. Ingulli E, Tejani A (1991) Incidence, treatment and outcome of recurrent focal segmental glomerulosclerosis post-transplantation in 42 allografts in children. A single-center experience. Transplantation 51: 401–405

    PubMed  Google Scholar 

  9. Boulton-Jones JM, Tulloch I, Dore B, Mc Lay A (1986) Changes in the glomerular capillary wall induced by lymphocyte products and serum of nephrotic patients. Clin Nephrol 20: 72–77

    Google Scholar 

  10. Zimmerman SW, Mann S (1984) Increased urinary protein excretion in the rat produced by serum from a patient with recurrent focal glomerular sclerosis after renal transplantation. Clin Nephrol 22: 32–38

    PubMed  Google Scholar 

  11. Lagrue G, Xhéneumont S, Branellec A, Hirbec G, Weil B (1975) A vascular permeability factor elaborated from lymphocytes. Demonstration in patients with nephrotic syndrome. Biomedicine 23: 37–40

    PubMed  Google Scholar 

  12. Franco A, Peres R, Anaya F, Niembro E, Valderrabano B (1987) Spontaneous remission of proteinuria in recurrent focal glomerulosclerosis. Reappraisal of plasma exchange treatment. Clin Nephrol 28:158–160

    Google Scholar 

  13. Habib R, Gagnadoux MF, Broyer M (1987) Recurrent glomerulonephritis in transplanted children. Contrib Nephrol 55: 123–125

    PubMed  Google Scholar 

  14. Dantal J, Baatard M, Hourmant M, Cantarovich D, Buzelin F, Soulillou JP (1991) Recurrent nephrotic syndrome following renal transplantation in patients with focal glomerulosclerosis. A one-center study of plasma exchange effects. Transplantation 52: 827–831

    PubMed  Google Scholar 

  15. Pinto J, Lacerda G, Cameron JS, Turner DR, Bewick M, Ogg CS (1981) Recurrence of focal segmental glomerulosclerosis in renal allografts. Transplantation 32: 83–89

    PubMed  Google Scholar 

  16. Verani RR, Hawkins EP (1986) Recurrent focal segmental glomerulosclerosis. Am J Nephrol 6: 263–270

    PubMed  Google Scholar 

  17. Munoz J, Sanchez R, Perez Garcia R, Anaya F, Valderrabano F (1985) Recurrent focal segmental glomerulosclerosis in renal transplants. Proteinuria relapsing following plasma exchange. Clin Nephrol 24: 213–214

    Google Scholar 

  18. Zimmerman SW (1985) Plasmapheresis and dipyridamole for recurrent glomerulosclerosis. Nephron 40: 241–245

    PubMed  Google Scholar 

  19. Laufer J, Ettenger RB, Ho WG, Cohen AH, Marik JL, Fine RN (1988) Plasma exchange for recurrent nephrotic syndrome following renal transplantation. Transplantation 46: 540–542

    PubMed  Google Scholar 

  20. Niaudet P, Broyer M, Habib R (1991) Treatment of idiopathic nephrotic syndrome with cyclosporin A in children. Clin Nephrol 35 [Suppl 1]: S31-S36

    PubMed  Google Scholar 

  21. Cattran DC (1988) Effect of cyclosporin on active Heymann's nephritis. Nephron 48: 142–148

    PubMed  Google Scholar 

  22. Borel JF (1991) Mechanism of action of cyclosporin A and rationale for use in nephrotic syndrome. Clin Nephrol 35 [Suppl 1]: S23-S30

    PubMed  Google Scholar 

  23. Pirson Y, Squifflet JP, Morbaix E, Alexandre GPJ, Ypersele de Strihou C van (1986) Recurrence of focal glomerulosclerosis despite cyclosporin treatment after renal transplantation. BMJ 292: 1336

    Google Scholar 

  24. Vincenti F, Biava C, Tomlanovitch S, Amend WJC Jr, Garovoy M, Melzer J, Feduska N, Salvatierra O Jr (1989) Inability of cyclosporine to completely prevent the recurrence of focal glomeruloselerosis after kidney transplantation. Transplantation 47: 595–598

    PubMed  Google Scholar 

  25. Tejani A, Butt K, Trachtwan H, Suthanthiran M, Rosenthal CJ, Khawar MR (1987) Cyclosporine induced remission of relapsing nephrotic syndrome in children. J Pediatr 111: 1056–1062

    PubMed  Google Scholar 

  26. Geary DF, Farine M, Thorner P, Baumal R (1984) Response to cyclophosphamide in steroid-resistant focal-segmental glomerulosclerosis: a reappraisal. Clin Nephrol 22: 109–113

    PubMed  Google Scholar 

  27. Mendoza SA, Reznik VM, Griswold WR, Krensky AM, Yorgin PD, Tune BM (1990) Treatment of steroid-resistant focal-segmental glomerulosclerosis with pulse methyl prednisolone and alkylating agents. Pediatr Nephrol 4: 303–307

    PubMed  Google Scholar 

  28. Korbet SM, Schwartz MM, Lewis EJ (1988) Recurrent nephrotic syndrome in renal allografts. Am J Kidney Dis 11: 270–276

    PubMed  Google Scholar 

  29. Mc Cauley J, Shapiro R, Scantlebury V, Gilboa N, Jordan M, Jensen C, Naik A, Tzakis A, Ellis D, Starzl TE (1991). FK 506 in the management of transplant-related nephrotic syndrome and steroidresistant nephrotic syndrome. Transplant Proc 23: 3354–3356

    PubMed  Google Scholar 

  30. Barrat TM, Cameron JS, Chantler C (1977) Controlled trial of azathioprine in treatment of steroid responsive nephrotic syndrome in childhood. Arch Dis Child 52: 462–465

    PubMed  Google Scholar 

  31. Rowe PC, Mc Lean RH, Ruley EJ, Salcedo JR, Baumgardner RA, Zaugg B, Mellits ED, De Angelis C (1990) Intravenous immunoglobulin in minimal change nephrotic syndrome: a cross over trial. Pediatr Nephrol 4: 32–35

    PubMed  Google Scholar 

  32. Torres VE, Velosa JA, Holley KE, Frohnert PP, Zincke H, Sterioff S (1984) Meclofenamate treatment of recurrent idiopathic nephrotic syndrome with focal segmental glomerulosclerosis after renal transplantation. Mayo Clin Proc 59: 146–152

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Unité de Néphrologie Pédiatrique, Hôpital Edouard Herriot, 69437, Lyon, France

    Pierre Cochat, Catherine Glastre, Barbara Tourniaire, Bernadette Parchoux & Louis David

  2. Université Claude Bernard, Lyon, France

    Pierre Cochat, Barbara Tourniaire, Bernadette Parchoux & Louis David

  3. Centre Régional de Transfusion Sanguine, Avenue du Vercors, Lyon, France

    Ali Kassir

  4. Laboratoire d'Anatomie Pathologie Rénale, Hôpital Edouard Herriot, Lyon, France

    Simone Colon

  5. Service d'Urologie et Chirurgie de la Transplantation, Hôpital Edouard Herriot, Lyon, France

    Xavier Martin

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  1. Pierre Cochat
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  2. Ali Kassir
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  3. Simone Colon
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  4. Catherine Glastre
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  7. Xavier Martin
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  8. Louis David
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Cochat, P., Kassir, A., Colon, S. et al. Recurrent nephrotic syndrome after transplantation: Early treatment with plasmaphaeresis and cyclophosphamide. Pediatr Nephrol 7, 50–54 (1993). https://doi.org/10.1007/BF00861567

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  • DOI: https://doi.org/10.1007/BF00861567

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