Abstract
We present the cases of two children with steroid-resistant nephrotic syndrome (SRNS) who were treated with rituximab (anti-CD20 monoclonal antibody). Both were resistant to conventional therapy, and renal biopsy showed focal segmental glomerulosclerosis (FSGS). Combination therapy with methylprednisolone pulse therapy and plasmapheresis was the only way to decrease proteinuria. However, the patients suffered severe reactions to steroid treatment. We therefore treated them with rituximab in a single dose of 375 mg/m2, which resulted in the rapid clearing of circulating CD19-positive B cells. One month after rituximab treatment, both achieved partial remission; one patient has maintained complete remission for 8 months, and the other relapsed 8 months after the first rituximab treatment with the recovery of peripheral B-cell counts and received a second rituximab treatment. She achieved complete remission 5 months after the second course and has maintained the remission for 2 months. We conclude that rituximab may be an effective treatment for refractory SRNS with FSGS.
References
Gipson DS, Gibson K, Gipson PE, Watkins S, Moxey-Mims M (2003) Therapeutic approach to FSGS in children. Pediatr Nephrol 22:28–36
Dantal J, Bigot E, Bogers W, Testa A, Kriaa F, Jacques Y, Hurault de Ligny B, Niaudet P, Charpentier B, Soulillou JP (1994) Effect of plasma protein adsorption on protein excretion in kidney-transplant recipients with recurrent nephrotic syndrome. N Engl J Med 330:7–14
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
Salama AD, Pusey CD (2006) Drug insight: rituximab in renal disease and transplantation. Nat Clin Pract Nephrol 2:221–230
Remuzzi G, Chiurchiu C, Abbate M, Brusegan V, Bontempelli M, Ruggenenti P (2002) Rituximab for idiopathic membranous nephropathy. Lancet 360:923–924
Benz K, Dotsch J, Rascher W, Stachel D (2004) Change of the course of steroid-dependent nephrotic syndrome after rituximab therapy. Pediatr Nephrol 19:794–797
Nozu K, Iijima K, Fujisawa M, Nakagawa A, Yoshikawa N, Matsuo M (2005) Rituximab treatment for posttransplant lymphoproliferative disorder (PTLD) induces complete remission of recurrent nephrotic syndrome. Pediatr Nephrol 20:1660–1663
Pescoviz MD, Book BK, Sider RA (2006) Resolution of recurrent focal segmental glomerulosclerosis proteinuria after rituximab treatment. N Engl J Med 354:1961–1963
Gilbert RD, Hulse E, Rigden S (2006) Rituximab therapy for steroid-dependent minimal change nephrotic syndrome. Pediatr Nephrol 21:1698–1700
Westphal S, Hansson S, Mjornstedt L, Molne J, Swerkersson S, Friman S (2006) Early recurrence of nephrotic syndrome (immunoglobulin M nephropathy) after renal transplantation successfully treated with combinations of plasma exchanges, immunoglobulin, and rituximab. Transplant Proc 38:2659–2660
Francois 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
Smith GC (2007) Is there a role for rituximab in the treatment of idiopathic childhood nephrotic syndrome? Pediatr Nephrol 22:893–898
Bagga A, Sinha A, Moudgil A (2007) Rituximab in patients with the steroid-resistant nephrotic syndrome. N Engl J Med 356:2751–2752
Hariharan S, Peddi VR, Savin VJ, Johnson CP, First MR, Roza AM, Adams MB (1998) Recurrent and de novo renal diseases after renal transplantation: a report from the renal allograft disease registry. Am J Kidney Dis 31:928–931
Artero ML, Sharma R, Savin VJ, Vincenti F (1994) Plasmapheresis reduces proteinuria and serum capacity to injure glomeruli in patients with recurrent focal glomerulosclerosis. Am J Kidney Dis 23:574–581
Sharma M, Sharma R, McCarthy ET, Savin VJ (1999) “The FSGS factor:” enrichment and in vivo effect of activity from focal segmental glomerulosclerosis plasma. J Am Soc Nephrol 10:552–561
Sharma M, Sharma R, McCarthy ET, Savin VJ (2004) The focal segmental glomerulosclerosis permeability factor: biochemical characteristics and biological effects. Exp Biol Med 229:85–98
Porakishvili N, Mageed R, Jamin C, Pers JO, Kulikova N, Renaudineau Y, Lydyard PM, Youinou P (2001) Recent progress in the understanding of B-cell functions in autoimmunity. Scand J Immunol 54:30–38
Roll P, Palanichamy A, Kneitz C, Dorner T, Tony HP (2006) Regeneration of B cell subsets after transient B cell depletion using anti-CD20 antibodies in rheumatoid arthritis. Arthritis Rheum 54:2377–2386
Kimby E (2005) Tolerability and safety of rituximab (MabThera). Cancer Treat Rev 31:456–473
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Nakayama, M., Kamei, K., Nozu, K. et al. Rituximab for refractory focal segmental glomerulosclerosis. Pediatr Nephrol 23, 481–485 (2008). https://doi.org/10.1007/s00467-007-0640-x
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00467-007-0640-x