Unsuccessful Treatment with Abatacept in Recurrent Focal Segmental Glomerulosclerosis after Kidney Transplantation

Abstract

Recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation occurs in up to 20-50% of FSGS patients and is associated with inferior allograft survival. Treatment of both primary FSGS as well as recurrent FSGS after transplantation with plasma exchange and immunosuppression is often unsuccessful and remains a major challenge as the disease still leads to end-stage renal disease and decreased graft survival. Previous case reports have described patients with recurrent FSGS who were successfully treated with a B7-1 inhibitor (abatacept) inducing partial or complete remission. The rational basis for believing in abatacept as a new therapeutic drug for the treatment of FSGS is the study by Yu et al. [N Engl J Med 2013;369: 2416-2423] showing B7-1 in immunostainings of the podocytes. The authors speculated that B7-1 immunostaining of renal biopsies might identify a subgroup of patients who would benefit from abatacept treatment. We present a case with recurrent FSGS after renal transplantation. The patient was unsuccessfully treated with B7-1 inhibitors. Although the patient was treated with abatacept 10 mg/kg body weight twice, the proteinuria and decreased graft function remained unchanged, and he never reached remission.

Keywords: Abatacept; B7-1 inhibitor; Focal segmental glomerulosclerosis; Recurrent focal segmental glomerulosclerosis.

Fig. 1

Light microscopy of the renal biopsy showing focal segmental glomerulosclerosis. Inset Electron microscopy of the renal biopsy showing podocyte foot process fusion.

Fig. 2

Time line of albuminuria and plasma creatinine in relation to therapeutic interventions. RAS, renin-angiotensin system inhibitor; PE, plasma exchange.