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Abstract

Focal segmental glomerulosclerosis is a common cause of end-stage renal disease in children. Focal segmental glomerulosclerosis recurrence in renal transplants is a challenging disease, and can cause graft dysfunction and loss. Different therapies exist with varying responses, from complete remission to resistance to all modes of treatment. Abatacept was recently introduced as a treatment for primary focal segmental glomerulosclerosis in native kidneys and in recurrent disease after transplant. We present a pediatric case with immunosuppression-resistant primary NPHS2-negative focal segmental glomerulosclerosis recur-rence after renal transplant. The standard therapy for recurrent focal segmental glomerulosclerosis (rituximab, plasmapheresis, highdose cyclosporine, and corticosteroids) was tried but failed to induce remission. Abatacept (10 mg/kg) was given at 0, 2, and 4 weeks (total, 3 doses) with no good response. We conclude that abatacept may work in patients with B7-1-positive focal segmental glomerulosclerosis recurrence and its efficacy is uncertain in disease with B7-1-negative or unknown staining status.

Update on the treatment of focal segmental glomerulosclerosis in renal transplantation

“…Garin et al [105] Abatacept (1 or [106] Abatacept (3 doses; 10 mg/kg) 1 patient (< 18 yr) No response Grellier et al [107] Belatacept (days 1, 15, 30 and monthly thereafter, 5 mg/kg) had achieved sustained remission. In a second series, remission was induced in 13/16 patients (81%), which also included PE sessions for 4 of the cases; CyA doses were from 6 to 25 mg/kg per day [46] .…”

“…Nevertheless, other studies of patients with FSGS recurrence have shown a slight/absent response after treatment with CTLA4-Ig [104][105][106][107] , despite the fact that in some of these cases belatacept (able to bind B71 with an higher affinity than abatacept) was adopted.…”

Recurrence of primary glomerulonephritis: Review of the current evidence

“…A case report recorded a complete remission using the T-cell costimulatory protein B7-1 blocker abatacept, which was not confirmed by others[ 145 - 147 ].…”

Monoclonal antibodies for renal diseases: current concepts and ongoing treatments

“…Some evidence and case reports highlighted that abatacept may block the progression of FSGS [44]. In this study, Abatacept induced partial or complete remission of proteinuria in a group of five patients B7-1-positive at glomerular level, suggesting that B7-1 may be a useful biomarker for the treatment of some glomerulopathies.…”