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Kidney Week

Abstract: FR-PO1064

Ravulizumab: The Allograft's Guardian in Atypical Hemolytic Uremic Syndrome, a Case Report

Session Information

Category: Transplantation

  • 2102 Transplantation: Clinical

Authors

  • Dweekat, Mo'tasem Zuhier (M. R.), University Hospitals Health System, Cleveland, Ohio, United States
  • Yerneni, Himabindu, University Hospitals Health System, Cleveland, Ohio, United States
  • Jittirat, Arksarapuk, University Hospitals Health System, Cleveland, Ohio, United States
  • Balabhadrapatruni, Krishna P., University Hospitals Health System, Cleveland, Ohio, United States
  • Boonpheng, Boonphiphop, University Hospitals Health System, Cleveland, Ohio, United States
Introduction

Atypical Hemolytic Uremic Syndrome (aHUS) is a rare disease that involves renal impairment and is caused by complement dysregulation. Ravulizumab (RAV) blocks Complement C5 and is approved for the treatment of aHUS. As a relatively new medication, it is less well studied in the transplant setting compared to Eculizumab (ECU). In a study of 29 kidney transplant recipients (25 of whom are adults) switched from ECU to RAV, Gaeckler et al. reported no episodes of rejection or graft failure.

Case Description

A 24-year-old male with history of ESRD due to aHUS underwent deceased donor kidney transplant at age 3 which failed 7 months later due to recurrence of aHUS, underwent a living donor kidney transplant at age 9 which failed 1 week later due to septic shock. The patient did not receive anti-complement therapy with the previous kidney transplants as only around the age of 15 was he diagnosed with aHUS due to CFH mutation. Patient eventually undergoes preparation with RAV then a Deceased donor kidney transplant 1 year later. 3 months following transplantation he is found to have acute cellular rejection successfully treated with steroid pulse. Now, 2.5 years following the last transplant, serum creatinine is stable at 1.3 mg/dl with an eGFR of 75. The last kidney biopsy performed 7 months following transplantation showed mild Interstitial fibrosis & Tubular atrophy, with no evidence of TMA or aHUS.

Discussion

RAV, is a monoclonal antibody that was re-engineered from ECU to extend its half-life and allow for longer dosing intervals. It has FDA approval for Myasthenia Gravis, Neuromyelitis Optica Spectrum Disorders, Paroxysmal Nocturnal Hemoglobinuria, and aHUS. In a phase 3 trial, Rondeau et al. reported achieving a complete TMA response in 53.6% of adult participants with aHUS, comparable to ECU use for the same indication. RAV was generally well tolerated in previous reports. Due to its mechanism of action, the risk of serious infections with encapsulated bacteria remains a concern. Vaccination against meningococcus is recommended prior to initiation of RAV. In this report, maintenance of the 3rd allograft was only achieved following use of RAV without exposure to ECU. Maintenance RAV is non-inferior and can be used as initiation medication for transplant immunosuppression in aHUS.

Digital Object Identifier (DOI)