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  • Title: Remission of steroid-resistant nephrotic syndrome due to focal and segmental glomerulosclerosis using rituximab.
    Author: Suri M, Tran K, Sharma AP, Filler G, Grimmer J.
    Journal: Int Urol Nephrol; 2008; 40(3):807-10. PubMed ID: 18491215.
    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.
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