Kidney Medicine (May 2019)

Monoclonal IgG4/2κ Deposition Following Eculizumab Therapy for Recurrent Atypical Hemolytic Uremic Syndrome in Kidney Transplantation

  • Priyamvada Singh,
  • Hui Chen,
  • Craig E. Gordon,
  • Sandeep Ghai,
  • J. Mark Sloan,
  • Karen Quillen,
  • Sara Moradi,
  • Vipul Chitalia,
  • Amitabh Gautam,
  • Joel Henderson,
  • Jean M. Francis

Journal volume & issue
Vol. 1, no. 3
pp. 139 – 143

Abstract

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Eculizumab is an emerging therapy for atypical hemolytic uremic syndrome (aHUS). Early identification and treatment of recurrent aHUS after kidney transplantation requires a high clinical suspicion but results in improved graft function and patient outcome. We present a patient who developed recurrent aHUS after kidney transplantation that responded to eculizumab therapy. A kidney biopsy was performed to confirm resolution of thrombotic microangiopathy 8 weeks after eculizumab treatment initiation and revealed no features of thrombotic microangiopathy. Instead, the biopsy revealed monoclonal immunoglobulin G (IgG)4/2κ deposition in the glomerular tufts, vasculature, and atrophic tubular basement membranes. IgG4/2κ deposits are a rare pathologic finding following eculizumab therapy, and the long-term effect of these deposits on kidney function remains unknown. Index Words: Eculizumab, aHUS, thrombotic microangiopathy