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Author Spotlight: Investigating the Potential of Chinese Herbal Medicinal Active Dioscin in Treating IgA Nephropathy
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Monoclonal IgG4/2κ Deposition Following Eculizumab Therapy for Recurrent Atypical Hemolytic Uremic Syndrome in Kidney

Priyamvada Singh1,2, Hui Chen3, Craig E Gordon1

  • 1Renal Section, Department of Medicine, Boston University School of Medicine, Boston, MA.

Kidney Medicine
|August 1, 2020
PubMed

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.

Keywords:
EculizumabaHUSthrombotic microangiopathy

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