Rituximab in post-transplant pediatric recurrent focal segmental glomerulosclerosis

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.

Recurrent focal segmental glomerulosclerosis after kidney transplantation

“…Only one study reported significant adverse effects from rituximab. While the effects reported were serious, only 7.5 % of patients treated had an adverse reaction, and all of these were within the same study [78].…”

“…Since the year 2000, numerous case reports and small studies of the efficacy of rituximab in treating FSGS have been published [62][63][64][65][66][67][68][69][70][71][72][73][74][75][76][77][78]. The larger case series are summarized in Table 3.…”

Update on the treatment of focal segmental glomerulosclerosis in renal transplantation

“…Kumar et al [117] observed a significant rate of severe complications (3/8 patients), ranging from Rituximab ® -associated lung injury, acute tubular necrosis, and central nervous system malignancy.…”

Efficacy of the Combination of Immunoadsorption and Rituximab for Treatment in a Case of Severe Focal and Segmental Glomerulosclerosis Recurrence after Renal Transplantation

“…The dosage of Rituximab in previous studies was between 1 and 4 doses [17, 18]. In our case, the aggressive recurrence along with the patient’s tolerance of the treatment, led us to administer 5 doses, although 3 or 4 doses may have been enough.…”