FSGS is the most common glomerular cause of ESKD in pediatric patients. The risk of recurrence after kidney transplant (KT) is 30-60%, and half of patients with recurrence experience graft loss. The optimal treatment of recurrent FSGS has not been established. We retrospectively examined our center's experience treating patients aged 1-20 years receiving KT for a diagnosis of Primary FSGS from January 2008 to November 2017. No pre-KT plasmapheresis (PP) was performed. Starting mid-2014, patients with this diagnosis were given one pre-KT dose of Rituximab (unless receiving alemtuzumab for high risk status) in addition to basiliximab as part of their induction therapy. Maintenance immunosuppression included MMF and tacrolimus, without prednisone unless a rejection episode occurred.

Over this 10 year period, 27 patients (13 living donor [LD]) with Primary FSGS were transplanted at the ages of 5 to 20 years. 7 (26%) had recurrence with initial graft function. Recurrence in those receiving pre-KT Rituximab was 2/8 (25%). Of the patients who had recurrent disease, 4/7 received LD kidneys (57%), as compared to 9/20 (45%) of those who did not recur. The ages at KT of those who did not recur were all ≥13 years. In contrast, the patients with recurrent disease were 5, 5, 6, 8, 11, 15, and 15 years old. Treatment for patients with recurrence included PP therapy (3/week until weaned with improvement in proteinuria) until either remission or graft loss. After 2012, 4 doses of Rituximab were also used. ACE-I +/- ARB therapy was also titrated in most after good graft function was established.

6 patients (86%) achieved full remission of their first recurrence. 5/6 patients completed their full PP course in our center. For these 5 patients, an average of 50 PP sessions (range 22-61) were required over an average of 141 days (range 54-193). 3 of these patients received Rituximab in addition to PP. Two of these patients had second recurrences 4-6 years after KT, with remission induced by 4 Rituximab doses plus intensification of ACE-I/ARB therapy. One patient failed to reach remission of initial recurrence and had graft failure requiring return to dialysis 5 months post-KT.

In summary, post-KT PP was effective in the majority of pediatric recipients with recurrent FSGS. Rituximab use pre- or post-KT for initial recurrence is of unclear additional benefit, and larger randomized studies are necessary to evaluate this question.

CITATION INFORMATION: Bobrowski A., Matossian D. Recurrence of Focal Segmental Glomerulosclerosis (FSGS) and Response to Prolonged Plasmapheresis Therapy: A Single Center Pediatric Experience Am J Transplant. 2017;17 (suppl 3).

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