Abstract
Treatment of steroid-resistant nephrotic syndrome (SRNS) remains a challenge to pediatric nephrologists. Recently, intravenous cyclophosphamide (IV-CPM) infusion was shown to be effective, safe, and economical for the treatment of SRNS, particularly minimal change disease (MCD), as it results in more sustained remissions, longer periods without proteinuria, and fewer significant side effects at a lower cumulative dose. A prospective study was conducted to evaluate IV-CPM infusions in the management of children with SRNS secondary to MCD or IgM nephropathy. Five patients with SRNS (4 IgM nephropathy and 1 MCD) received six monthly IV-CPM infusions at a dose of 500 mg/m2. No patient achieved complete or sustained remission. Three patients attained partial remission, which was not sustained for more than 1 month post therapy. One patient progressed rapidly to end-stage renal disease during treatment. Side effects included vomiting in four patients and alopecia in one patient. Conclusion: IV-CPM pulse therapy at a dose of 500 mg/m2 is unsuccessful in obtaining complete or sustained remission in children with SRNS secondary to IGM nephropathy or MCD. Further randomized controlled studies with higher doses are required.
Similar content being viewed by others
References
The International Study of Kidney Disease in Children (1981) The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. J Pediatr 98:561–564
McBryde KD, Kershaw DB, Smoyer WE (2001) Pediatric steroid-resistant nephrotic syndrome. Curr Probl Pediatr Adolesc Health Care 31:280–307
International Study of Kidney Disease in Children (1978) Nephrotic syndrome in children: prediction of histopathology from clinical and laboratory characteristics at time of diagnosis. Kidney Int 13:159–165
Sorof JM, Hawkins EP, Brewer ED, Boydstun II, Kale AS, Powell DR (1998) Age and ethnicity affect the risk and outcome of focal segmental glomerulosclerosis. Pediatr Nephrol 12:764–768
Niaudet P (1994) Treatment of childhood steroid-resistant idiopathic nephrosis with a combination of cyclosporine and prednisone. J Pediatr 125:981–986
Vehaskari VM (1999) Treatment practices of FSGS among North American pediatric nephrologists. Pediatr Nephrol 13:301–303
Burgess E (1999) Management of focal segmental glomerulosclerosis: evidence-based recommendations. Kidney Int [Suppl 70]:S26–S32
Lieberman KV, Tejani A (1996) A randomized double-blind placebo-controlled trial of cyclosporine in steroid-resistant idiopathic focal segmental glomerulosclerosis in children. J Am Soc Nephrol 7:56–63
Grupe WE, Heymann W (1966) Cytotoxic drugs in steroid-resistant renal disease. Alkylating and antimetabolic agents in the treatment of nephrotic syndrome, lupus nephritis, chronic glomerulonephritis, and purpura nephritis in children. Am J Dis Child 112:448–458
Bargman JM (1999) Management of minimal lesion glomerulonephritis: evidence-based recommendations. Kidney Int [Suppl 70]:S3–S16
Gulati S, Kher V (2000) Intravenous pulse cyclophosphamide--a new regime for steroid resistant focal segmental glomerulosclerosis. Indian Pediatr 37:141–148
Elhence R, Gulati S, Kher V, Gupta A, Sharma RK (1994) Intravenous pulse cyclophosphamide—a new regime for steroid-resistant minimal change nephrotic syndrome. Pediatr Nephrol 8:1–3
Niaudet P, Gagnadoux MF, Broyer M (1998) Treatment of childhood steroid-resistant idiopathic nephrotic syndrome. Adv Nephrol Necker Hosp 28:43–61
Al-Eisa A, Carter JE, Lirenman DS, Magil AB (1996) Childhood IgM nephropathy: comparison with minimal change disease. Nephron 72:37–43
Donia AF, Sobh MA, Moustafa FE, Bakr MA, Foda MA (2000) Clinical significance and long-term evolution of minimal change histopathologic variants and of IGM nephropathy among Egyptians. J Nephrol 13:275–281
Abdurrahman MB, Elidrissy AT, Mahmoud K, Rasheed S al, Mugeirin M al (1993) Failure of clinical and laboratory characteristics to differentiate mesangial proliferative from minimal-change nephrotic syndrome. Acta Paediatr 82:579–581
Zeis PM, Kavazarakis E, Nakopoulou L, Moustaki M, Messaritaki A, Zeis MP, Nicolaidou P (2001) Glomerulopathy with mesangial IgM deposits: long-term follow up of 64 children. Pediatr Int 43:287–292
Kirpekar R, Yorgin PD, Tune BM, Kim MK, Sibley RK (2002) Clinicopathologic correlates predict the outcome in children with steroid-resistant idiopathic nephrotic syndrome treated with pulse methylprednisolone therapy. Am J Kidney Dis 39:1143–1152
Kopolovic J, Shvil Y, Pomeranz A, Ron N, Rubinger D, Oren R (1987) IgM nephropathy: morphological study related to clinical findings. Am J Nephrol 7:275–280
Garcia DM, Gomez-Morales M, Cortes V, Aguayo ML, Gigosos RL, Lardelli P, Navas A, Aneiros J, Aguilar D (1993) Mononuclear cell subsets in IgM mesangial proliferative glomerulonephritis. A comparative study with minimal change nephrotic syndrome and immunonegative mesangial proliferative glomerulonephritis. Nephron 65:215–221
Bajpai A, Bagga A, Hari P, Dinda A, Srivastava RN (2003) Intravenous cyclophosphamide in steroid-resistant nephrotic syndrome. Pediatr Nephrol 18:351–356
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Alshaya, H.O., Al-Maghrabi, J.A. & Kari, J.A. Intravenous pulse cyclophosphamide—is it effective in children with steroid-resistant nephrotic syndrome?. Pediatr Nephrol 18, 1143–1146 (2003). https://doi.org/10.1007/s00467-003-1279-x
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00467-003-1279-x