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Kidney Week

Abstract: SA-PO384

Using Eculizumab for Immediate Recurrence of ANCA Vasculitis in a Pediatric Kidney Transplant

Session Information

  • Pediatric Nephrology - III
    November 04, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

  • 1900 Pediatric Nephrology

Authors

  • Lomanta, Francis Vincent, University of Utah Health, Salt Lake City, Utah, United States
  • Sherbotie, Joseph R., University of Utah Health, Salt Lake City, Utah, United States
  • Peterson, Caitlin G., University of Utah Health, Salt Lake City, Utah, United States

Group or Team Name

  • University of Utah, Division of Pediatric Nephrology.
Introduction

ANCA associated vasculitis (AAV) is associated with poor renal outcomes with 20-30% of patients developing chronic kidney disease and up to 20-35% progressing to end-stage kidney disease. Recent advances have led to increasing interest towards complement inhibition as a potential treatment for AAV. We present a case of disease recurrence after pediatric kidney transplantation and the novel use of Eculizumab to salvage graft function.

Case Description

A 5-year-old Hispanic male with ESKD due to myeloperoxidase antibody positive ANCA vasculitis developed disease recurrence post-kidney transplant. Pre-transplant disease control required multiple rounds of high dose steroids, cyclophosphamide, azathioprine, and rituximab. Transplant induction included thymoglobulin and pulse-dose steroids with rapid taper. Mycophenolate (MMF) and tacrolimus (TAC) was started on POD 1 and 2 respectively. He developed gross hematuria on POD 5 with doubling of his serum creatinine (sCr) on POD 8. Kidney biopsy was done on POD 14 which showed pauci-immune glomerulonephritis and acute tubular necrosis without rejection. He was treated with plasmapheresis, pulse-dose steroids, and a dose of rituximab. Compassionate use of a C5a receptor inhibitor was sought and denied. Eculizumab, a C5 inhibitor was given instead on POD 28. With MMF, TAC, Eculizumab and steroid taper, his sCr improved. Two months later, he was transitioned to Ravulizumab. His kidney function remains stable and he continues to receive infusions without concerns of disease recurrence or serious infections.

Discussion

Previous reports of Eculizumab use for AAV led to our decision to use it for our patient with historically difficult to control AAV. This case illustrates a rare presentation of immediate disease recurrence of AAV in a pediatric kidney transplant and the successful use of C5 blockade. No specific treatment guidelines exist for childhood AAV, currently highlighting the need for further studies to evaluate efficacy and safety of complement inhibitors as treatment for childhood AAV.