Rituximab in post-transplant pediatric recurrent focal segmental glomerulosclerosis.
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.
Weill Cornell Medical College, Helmsley Towers 3, Box 176, New York, NY 10021, USA. juk2013@med.cornell.edu
Juhi
J
Kumar
Ibrahim F
IF
Shatat
Amy L
AL
Skversky
Robert P
RP
Woroniecki
Marcela
M
Del Rio
Eduardo M
EM
Perelstein
Valerie L
VL
Johnson
Shefali
S
Mahesh
D000293:Adolescent; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000188:drug therapy* / Q000628:therapy; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000601:surgery; D016030:Kidney Transplantation; D008297:Male; D010956:Plasmapheresis; D011507:Proteinuria / Q000209:etiology; D012008:Recurrence; D012189:Retrospective Studies; D000069283:Rituximab; D016896:Treatment Outcome
D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab