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Rituximab in post-transplant pediatric recurrent focal segmental glomerulosclerosis

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Abstract

Background

Focal segmental glomerulosclerosis (FSGS) recurs in 20–40 % of allografts. Plasmapheresis (TPE) has been one of the mainstays of treatment with variable results. Rituximab (RTX), a monoclonal antibody to the protein CD20, is being used for treatment of recurrent FSGS (recFSGS) but pediatric experience is limited.

Methods

We conducted a retrospective review of eight patients with recFSGS, treated with RTX (1–4 doses) after having minimal response to TPE. Complete response was defined as a decrease in urine protein creatinine ratio (Up/c) to less than 0.2 and partial response was a decrease in Up/c ratio by 50 % of baseline and in the sub-nephrotic range (U p/c <2).

Results

Complete response was seen in two of eight patients, and partial response was seen in four of eight patients. Two patients had no response. At last follow-up, all the partial responders had sub-nephrotic range proteinuria (Up/c ratios ranging from 0.29 to 1.6). Delayed response, up to 9 months post-RTX, was also seen in some of the patients. Significant complications such as rituximab-associated lung injury (RALI), acute tubular necrosis, and central nervous system(CNS) malignancy were also observed in our case series.

Conclusions

Rituximab can be used with caution as a treatment for recFSGS. Efficacy is variable from none to complete response. Even partial reduction in proteinuria is of benefit in prolonging the life of the allograft. Long-term, multicenter studies are needed to prove its sustained efficacy in those who respond and to monitor for serious adverse effects.

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Authors and Affiliations

  1. Weill Cornell Medical College, Helmsley Towers 3, Box 176, New York, NY, 10021, USA

    Juhi Kumar, Eduardo M. Perelstein & Valerie L. Johnson

  2. MUSC Children’s Hospital, Charleston, SC, USA

    Ibrahim F. Shatat

  3. Children’s Hospital at Montefiore, Bronx, NY, USA

    Amy L. Skversky, Robert P. Woroniecki & Marcela Del Rio

  4. Akron Children’s Hospital, Akron, OH, USA

    Shefali Mahesh

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  1. Juhi Kumar
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  2. Ibrahim F. Shatat
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Correspondence to Juhi Kumar.

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Kumar, J., Shatat, I.F., Skversky, A.L. et al. Rituximab in post-transplant pediatric recurrent focal segmental glomerulosclerosis. Pediatr Nephrol 28, 333–338 (2013). https://doi.org/10.1007/s00467-012-2314-6

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  • DOI: https://doi.org/10.1007/s00467-012-2314-6

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