Rituximab in post-transplant pediatric recurrent focal segmental glomerulosclerosis

Juhi Kumar, Ibrahim F. Shatat, Amy L. Skversky, Robert P. Woroniecki, Marcela Del Rio, Eduardo M. Perelstein, Valerie L. Johnson, Shefali Mahesh

Research output: Contribution to journalArticle

25 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)333-338
Number of pages6
JournalPediatric Nephrology
Volume28
Issue number2
DOIs
StatePublished - Feb 2013

Fingerprint

Focal Segmental Glomerulosclerosis
Pediatrics
Transplants
Creatinine
Urine
Proteinuria
Allografts
Proteins
Plasmapheresis
Acute Lung Injury
Multicenter Studies
Necrosis
Therapeutics
Central Nervous System
Monoclonal Antibodies
Rituximab
Neoplasms

Keywords

  • Focal segmental glomerulosclerosis
  • Plasmapheresis
  • Proteinuria
  • Recurrent disease
  • Transplant

ASJC Scopus subject areas

  • Nephrology
  • Pediatrics, Perinatology, and Child Health

Cite this

Kumar, J., Shatat, I. F., Skversky, A. L., Woroniecki, R. P., Del Rio, M., Perelstein, E. M., ... Mahesh, S. (2013). Rituximab in post-transplant pediatric recurrent focal segmental glomerulosclerosis. Pediatric Nephrology, 28(2), 333-338. https://doi.org/10.1007/s00467-012-2314-6

Rituximab in post-transplant pediatric recurrent focal segmental glomerulosclerosis. / Kumar, Juhi; Shatat, Ibrahim F.; Skversky, Amy L.; Woroniecki, Robert P.; Del Rio, Marcela; Perelstein, Eduardo M.; Johnson, Valerie L.; Mahesh, Shefali.

In: Pediatric Nephrology, Vol. 28, No. 2, 02.2013, p. 333-338.

Research output: Contribution to journalArticle

Kumar, J, Shatat, IF, Skversky, AL, Woroniecki, RP, Del Rio, M, Perelstein, EM, Johnson, VL & Mahesh, S 2013, 'Rituximab in post-transplant pediatric recurrent focal segmental glomerulosclerosis', Pediatric Nephrology, vol. 28, no. 2, pp. 333-338. https://doi.org/10.1007/s00467-012-2314-6
Kumar, Juhi ; Shatat, Ibrahim F. ; Skversky, Amy L. ; Woroniecki, Robert P. ; Del Rio, Marcela ; Perelstein, Eduardo M. ; Johnson, Valerie L. ; Mahesh, Shefali. / Rituximab in post-transplant pediatric recurrent focal segmental glomerulosclerosis. In: Pediatric Nephrology. 2013 ; Vol. 28, No. 2. pp. 333-338.
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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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AU - Kumar, Juhi

AU - Shatat, Ibrahim F.

AU - Skversky, Amy L.

AU - Woroniecki, Robert P.

AU - Del Rio, Marcela

AU - Perelstein, Eduardo M.

AU - Johnson, Valerie L.

AU - Mahesh, Shefali

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N2 - 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.

AB - 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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