Abstract
We report a case of a 2-year-old boy with steroid- and ciclosporin (CsA)-resistant collapsing focal segmental glomerulosclerosis (FSGS) who went into complete remission with a combination of IV rituximab and methylprednisolone pulse therapy (MPT) while receiving oral CsA. He was initially treated with steroids including MPT, CsA, and plasmapheresis, but his massive proteinuria and severe systemic edema persisted. We treated him with four weekly doses of intravenous rituximab. After the second administration of rituximab, his proteinuria and systemic edema were dramatically improved, but hypoalbuminemia and proteinuria persisted. Eight months after the onset, he was re-treated with two courses of MPT. Thereafter, he finally went into complete remission 1 month after MPT. He continued in remission for 8 months with CsA, but then relapsed. However, he went into complete remission again with 60 mg/m2 of oral prednisolone without rituximab and since then, he has been in remission with CsA. This is the first report of the successful treatment of collapsing FSGS with rituximab. Thus, rituximab emerges as a new therapeutic option against refractory collapsing FSGS.
This is a preview of subscription content, log in via an institution to check access.
References
Detwiler RK, Falk RJ, Hogan SL, Jennette JC (1994) Collapsing glomerulopathy: a clinically and pathologically distinct variant of focal segmental glomerulosclerosis. Kidney Int 45:1416–1424
Singh HK, Baldree LA, McKenney DW, Hogan SL, Jennette JC (2000) Idiopathic collapsing glomerulopathy in children. Pediatr Nephrol 14:132–137
Bagga A, Sinha A, Moudgil A (2007) Rituximab in patients with the steroid-resistant nephrotic syndrome. N Engl J Med 356:2751–2752
Nakayama M, Kamei K, Nozu K, Matsuoka K, Nakagawa A, Sako M, Iijima K (2008) Rituximab for refractory focal segmental glomerulosclerosis. Pediatr Nephrol 23:481–485
Laurinavicius A, Hurwitz S, Rennke HG (1999) Collapsing glomerulopathy in HIV and non-HIV patients: a clinicopathological and follow-up study. Kidney Int 56:2203–2213
Suri M, Tran K, Sharma AP, Filler G, Grimmer J (2008) Remission of steroid-resistant nephrotic syndrome due to focal segmental glomerulosclerosis using rituximab. Int Urol Nephrol 40:807–810
Kimmel PL, Mishkin GJ, Umana WO (1996) Captoril and renal survival in patients with human immunodeficiency virus nephropathy. Am J Kidney Dis 28:202–208
Chun MJ, Korbet SM, Schwartz MM, Lewis EJ (2004) Focal segmental glomerulosclerosis in nephrotic adults: presentation, prognosis, and response to therapy of the histologic variants. J Am Soc Nephrol 15:2169–2177
Kemper MJ, Meyer-Jark T, Lilova M, Muller-Wiefel DE (2003) Combined T- and B-cell activation in childhood steroid-sensitive nephrotic syndrome. Clin Nephrol 60:242–247
Guigonis V, Dallocchio A, Baudouin V, Dehennault M, Camus CH, Afanetti M, Groothoff J, Llanas B, Niaudet P, Nivet H, Raynaud N, Taque S, Ronco P, Bouissou F (2008) Rituximab treatment for severe steroid- or cyclosporine- dependent nephrotic syndrome: a multicentric series of 22 cases. Pediatr Nephrol 23:1269–1279
Sharma AP, Filler G (2009) Role of mycophenolate mofetil in remission maintenance after a successful response to rituximab. Pediatr Nephrol 24:423–424
Acknowledgement
We acknowledge Dr. Satoshi Hisano, Assistant Professor of Pathology at Fukuoka University Faculty of Medicine, for his pathological advice and support.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kaito, H., Kamei, K., Kikuchi, E. et al. Successful treatment of collapsing focal segmental glomerulosclerosis with a combination of rituximab, steroids and ciclosporin. Pediatr Nephrol 25, 957–959 (2010). https://doi.org/10.1007/s00467-009-1410-8
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00467-009-1410-8