Skip to main content
SpringerLink
Log in

Remission of steroid-resistant nephrotic syndrome due to focal and segmental glomerulosclerosis using rituximab

  • Nephrology - Case Report
  • Published:
International Urology and Nephrology Aims and scope Submit manuscript

Abstract

Background

Therapeutic options are limited in cases of focal and segmental glomerulosclerosis (FSGS) that fail to respond to steroids and calcineurin inhibitors. We describe a case in which steroid-resistant nephrotic syndrome (SRNS) secondary to FSGS did not respond to conventional treatment, but was successfully treated with rituximab. Unlike previous reports in which rituximab was used in conjunction with a calcineurin inhibitor, we present the first case where rituximab was used as the sole therapeutic agent.

Case report

An 11-month-old boy presented with severe manifestations of primary nephrotic syndrome with a subsequent non-responsive steroid course. A renal biopsy confirmed FSGS, with normal staining for podocin and nephrin. Genetic studies for podocin were normal. The child developed ciclosporin-induced hemolytic uremic syndrome (HUS), and the response to plasma exchange, following ciclosporin withdrawal, was only temporary. A trial of a combination of mycophenolate and dexamethasone did not have any effect on proteinuria or fluid status. Four weekly rituximab infusions at 375 mg/m2/dose induced a complete remission without any adverse effects.

Conclusions

This case suggests that rituximab may be used as the sole therapeutic agent in the treatment of SRNS secondary to FSGS, especially in cases where calcineurin inhibitors are contraindicated.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

References

  1. Borges FF, Shiraichi L, da Silva MP, Nishimoto EI, Nogueira PC (2007) Is focal segmental glomerulosclerosis increasing in patients with nephrotic syndrome? Pediatr Nephrol 22:1309–1313

    Article  PubMed  Google Scholar 

  2. Filler G, Young E, Geier P, Carpenter B, Drukker A, Feber J (2003) Is there really an increase in non-minimal change nephrotic syndrome in children? Am J Kidney Dis 42:1107–1113

    Article  PubMed  Google Scholar 

  3. Cattran DC, Appel GB, Hebert LA et al (1999) A randomized trial of cyclosporine in patients with steroid-resistant focal segmental glomerulosclerosis. North America Nephrotic Syndrome Study Group. Kidney Int 56:2220–2226

    Article  PubMed  CAS  Google Scholar 

  4. Cattran DC (2003) Cyclosporine in the treatment of idiopathic focal segmental glomerulosclerosis. Semin Nephrol 23:234–241

    Article  PubMed  CAS  Google Scholar 

  5. Bhimma R, Adhikari M, Asharam K, Connolly C (2006) Management of steroid-resistant focal segmental glomerulosclerosis in children using tacrolimus. Am J Nephrol 26:544–551

    Article  PubMed  CAS  Google Scholar 

  6. Westhoff TH, Schmidt S, Zidek W, Beige J, Van der Giet M (2006) Tacrolimus in steroid-resistant and steroid-dependent nephrotic syndrome. Clin Nephrol 65:393–400

    PubMed  CAS  Google Scholar 

  7. Ciechanska E, Segal L, Wong H, Chretien C, Feber J, Filler G (2005) Plasma exchange using a continuous venovenous hemofiltration machine in children. Blood Purif 23:440–445

    Article  PubMed  Google Scholar 

  8. Multicenter clinical trial of focal glomerulosclerosis in children and young adults. http://www.niddk.nih.gov/patient/fsgs/fsgs.htm#3, http://grants.nih.gov/grants/guide/rfa-files/RFA-DK-02-013.html

  9. Pandirikkal VB, Jain M, Gulati S (2007) Tacrolimus-induced HUS: An unusual cause of acute renal failure in nephrotic syndrome. Pediatr Nephrol 22:298–300

    Article  PubMed  Google Scholar 

  10. Bagga A, Sinha A, Moudgil A (2007) Rituximab in patients with the steroid-resistant nephrotic syndrome. N Engl J Med 356:2751–2752

    Article  PubMed  CAS  Google Scholar 

  11. Benz K, Dötsch J, Rascher W, Stachel D (2004) Change of the course of steroid-dependent nephrotic syndrome after rituximab therapy. Pediatr Nephrol 19:794–797

    Article  PubMed  Google Scholar 

  12. Smith GC (2007) Is there a role for rituximab in the treatment of idiopathic childhood nephrotic syndrome? Pediatr Nephrol 22:893–898

    Article  PubMed  Google Scholar 

  13. Gilbert RD, Hulse E, Rigden S (2006) Rituximab therapy for steroid-dependent minimal change nephrotic syndrome. Pediatr Nephrol 21:1698–1700

    Article  PubMed  Google Scholar 

  14. Cattran DC, Wang MM, Appel G, Matalon A, Briggs W (2004) Mycophenolate mofetil in the treatment of focal segmental glomerulosclerosis. Clin Nephrol 62:405–411

    PubMed  CAS  Google Scholar 

  15. Savin VJ, Sharma R, Sharma M, McCarthy ET, Swan SK, Ellis E, Lovell H, Warady B, Gunwar S, Chonko AM, Artero M, Vincenti F (1996) Circulating factor associated with increased glomerular permeability to albumin in recurrent focal segmental glomerulosclerosis. N Engl J Med 334:878–883

    Article  PubMed  CAS  Google Scholar 

  16. Savin VJ, McCarthy ET, Sharma M (2003) Permeability factors in focal segmental glomerulosclerosis. Semin Nephrol 23:147–160

    Article  PubMed  CAS  Google Scholar 

  17. Salama AD, Pusey CD (2006) Drug insight: rituximab in renal disease and transplantation. Nat Clin Pract Nephrol 2:221–230

    Article  PubMed  CAS  Google Scholar 

  18. Pescovitz MD (2006) Rituximab, an anti-CD20 monclonal antibody: history and mechanism of action. Am J Transplant 6:859–866

    Article  PubMed  CAS  Google Scholar 

  19. Klastersky J (2006) Adverse effects of the humanized antibodies used as cancer therapeutics. Curr Opin Oncol 18:316–320

    Article  PubMed  CAS  Google Scholar 

  20. Rey J, Belmecheri N, Bouayed N, Ivanov V, Coso D, Gastaut JA, Bouabdallah R (2007) JC papovavirus leukoencephalopathy after first line treatment with CHOP and rituximab. Haematologica 92(10):e101

    Article  PubMed  CAS  Google Scholar 

  21. Freim Wahl SG, Folvik MR, Torp SH (2007) Progressive multifocal leukoencephalopathy in a lymphoma patient with complete remission after treatment with cytostatics and rituximab: case report and review of the literature. Clin Neuropathol 26(2):68–73

    PubMed  CAS  Google Scholar 

  22. Remuzzi G, Chiurchiu C, Abbate M, Brusegan V, Bontempelli M, Ruggenenti P (2002) Rituximab for idiopathic membranous nephropathy. Lancet 360:923–924

    Article  PubMed  CAS  Google Scholar 

  23. Cobo M, Hernández D, Rodriguez C, Pérez-Tamajón L (2006) Successful therapeutic use of rituximab in refractory membranous glomerulonephritis. Clin Nephrol 66:54–57

    PubMed  CAS  Google Scholar 

  24. François H, Daugas E, Bensman A, Ronco P (2007) Unexpected efficacy of rituximab in multirelapsing minimal change nephrotic syndrome in the adult: first case report and pathophysiological considerations. Am J Kidney Dis 49:158–161

    Article  PubMed  Google Scholar 

  25. Hofstra JM, Deegens JK, Wetzels JF (2007) Rituximab: effective treatment for severe steroid-dependent minimal change nephrotic syndrome? Nephrol Dial Transplant 22:2100–2102

    Article  PubMed  Google Scholar 

  26. Yang T, Nast CC, Vo A, Jordan SC (2008) Rapid remission of steroid and mycophenolate mofetil (mmf)-resistant minimal change nephrotic syndrome after rituximab therapy. Nephrol Dial Transplant 23:377–380

    Article  PubMed  Google Scholar 

  27. Salama AD, Pusey CD (2006) Drug insight: rituximab in renal disease and transplantation. Nat Clin Pract Nephrol 2:221–230

    Article  PubMed  CAS  Google Scholar 

  28. Sheashaa H, Mahmoud I, El-Basuony F, El-Husseini A, Hassan N, El-Baz M, Ahmed NS, Sobh M (2007) Does cyclosporine achieve a real advantage for treatment of idiopathic nephrotic syndrome in children? A long-term efficacy and safety study. Int Urol Nephrol 39:923–928

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

We thank Dr Paul Thorner, pathologist at the Hospital for Sick Children in Toronto for the podocin and nephrin stains as well as Dr Diane Hebert, pediatric nephrologist at the Hospital for Sick Children in Toronto for sharing the planned protocol for treatment with rituximab for children with primary FSGS.

Author information

Authors and Affiliations

  1. Department of Pediatrics, Children’s Hospital of Western Ontario, University of Western Ontario, 800 Commissioners Road East, London, ON, Canada, N6A 5W9

    Megha Suri, Ajay P. Sharma, Guido Filler & Joanne Grimmer

  2. McMaster University Undergraduate Medical Program, Hamilton, ON, Canada

    Kim Tran

Authors
  1. Megha Suri
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kim Tran
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ajay P. Sharma
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Guido Filler
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Joanne Grimmer
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Guido Filler.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Suri, M., Tran, K., Sharma, A.P. et al. Remission of steroid-resistant nephrotic syndrome due to focal and segmental glomerulosclerosis using rituximab. Int Urol Nephrol 40, 807–810 (2008). https://doi.org/10.1007/s11255-008-9393-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11255-008-9393-0

Keywords

Search

Navigation