Successful Preemptive Kidney Transplantation With Rituximab Induction in a Patient With Focal Segmental Glomerulosclerosis and Massive Nephrotic Syndrome: A Case Report.

Primary focal segmental glomerulosclerosis (FSGS) recurs in 30% of patients receiving their first kidney transplant and often leads to graft loss. In the past, patients with FSGS and overt nephrotic syndrome rarely underwent transplantation. Rituximab (RTX), an anti-CD20-specific monoclonal antibody, was previously reported to be a valuable option in treating relapsing FSGS after kidney transplantation. We report here the first successful kidney transplantation in a young patient with primary FSGS and massive nephrotic syndrome treated with RTX induction. The patient was a 24-year-old woman who had developed nephrotic syndrome at the age of 4 years. FSGS was confirmed by results of a kidney biopsy, with subsequent treatment with cyclosporine and steroids, without remission. She was referred for a preemptive, deceased donor kidney transplant despite proteinuria levels reaching ∼10 g/d. She received induction therapy with 2 doses of RTX (375 mg/m2 ) at days 0 and 7, followed by tacrolimus 5 mg twice daily, mycophenolate mofetil 500 mg twice daily, and steroids after transplantation. Immediate kidney graft function was observed, with no proteinuria since day 13 posttransplant. The pretransplant soluble urokinase-type plasminogen activator receptor serum concentration was 4550 pg/mL; it decreased to 2191 pg/mL at day 13 and was 2073 pg/mL at 6 months' posttransplant. Thirty months after transplantation, the patient's serum creatinine level is 0.8 mg/dL, and no proteinuria has been observed. Successful kidney transplantation in a patient with pretransplant overt nephrotic syndrome secondary to FSGS, using rituximab as an induction therapy, is possible. Further recommendations for transplantation in such patients, however, should be based on results from larger clinical trials.