What is the role of rituximab in the treatment of frequently relapsing nephrotic syndrome (FRNS)?

Updated: Mar 04, 2020
  • Author: Jerome C Lane, MD; Chief Editor: Craig B Langman, MD  more...
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Rituximab is a chimeric anti-CD20 antibody that results in depletion of B cells. Rituximab may be considered in children with SDNS or FRNS in whom other treatments have failed or those with cumulative toxicity of other steroid-sparing agents. The usual dosing schedule is 2-4 weekly doses of rituximab (375 mg/m2 per dose).

A study by Basu et al that included 176 pediatric patients with SDNS reported a higher 12-month relapse-free survival rate in the rituximab group than in the TAC group (90.0% vs 63.3%) as well as a lower 12-month cumulative corticosteroid dose in the rituximab group. [82]

Kamei et al examined the long-term outcomes and safety of rituximab treatment to prevent relapses of complicated FRNS and SDNS in 51 children and reported that 94% of patients in the study developed relapses during the observation period (median, 59 months); the 50% relapse-free survival was 261 days. Fifty-nine percent of the patients developed SDNS, 86% required re-administration of immunosuppressive agents, and 43% received additional rituximab treatment. [83]

In an open-label, randomized, controlled, noninferiority trial, 54 children with steroid- or CNI-dependent INS were randomized to either standard treatment (prednisone/CNI) or rituximab in addition to low-dose prednisone and a CNI. Three months after randomization, proteinuria was 70% lower in patients treated with rituximab than in those who received standard therapy; relapse occurred in 18.5% of the rituximab group and 48% of the standard treatment group. At 3 months, 63% of the rituximab group was drug free, compared with 4% of the standard treatment group. It was concluded that rituximab with low-dose prednisone and a CNI was noninferior to standard therapy in maintaining short-term remission in children with steroid- and CNI-dependent INS. [8]

A multicenter, randomized, controlled trail in 48 children with FRNS or SDNS demonstrated a significantly longer median relapse-free period for rituximab (267 days) than for placebo (101 days). [9]

The adverse effects of rituximab are rare but potentially serious. Risks include Pneumocystis jirovecii pneumonia, pulmonary fibrosis, and progressive multifocal leukoencephalopathy. [78]  

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