Journal
KIDNEY MEDICINE
Volume 1, Issue 3, Pages 139-143Publisher
ELSEVIER
DOI: 10.1016/j.xkme.2019.03.005
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Funding
- NHLBI NIH HHS [R01 HL132325] Funding Source: Medline
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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 immuno-globulin G (IgG)4/2 kappa deposition in the glomerular tufts, vasculature, and atrophic tubular basement membranes. IgG4/2 kappa deposits are a rare pathologic finding following eculizumab therapy, and the long-term effect of these deposits on kidney function remains unknown. (C) 2019 The Authors. Published by Elsevier Inc. on behalf of the National Kidney Foundation, Inc.
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