Background: The development of complement inhibitors has greatly improved the outcome of patients with atypical hemolytic uremic syndrome (aHUS), making kidney transplantation a more feasible option. Although prophylactic eculizumab therapy may prevent recurrent disease after transplantation, its necessity for all transplant recipients is debated.
Study design: A case series.
Setting & participants: Patients with aHUS who underwent living donor kidney transplantation after 2011 at 2 university centers, prospectively followed up with a protocol of eculizumab therapy limited to only recipients with documented posttransplantation recurrent thrombotic microangiopathy. In addition, the protocol emphasized lower target level tacrolimus and aggressive treatment of high blood pressure.
Outcomes: Recurrence of aHUS, kidney function, acute kidney injury.
Results: We describe 12 female and 5 male patients with a mean age of 47 years. 5 patients had lost a previous transplant due to aHUS recurrence. 16 patients carried a pathogenic or likely pathogenic variant in genes encoding complement factor H, C3, or membrane cofactor protein, giving a high risk for aHUS recurrence. Median follow-up after transplantation was 25 (range, 7-68) months. One patient had aHUS recurrence 68 days after transplantation, which was successfully treated with eculizumab. 3 patients were treated for rejection and 2 patients developed BK nephropathy. At the end of follow-up, median serum creatinine concentration was 106 (range, 67-175) μmol/L and proteinuria was negligible.
Limitations: Small series and short duration of follow-up.
Conclusions: Living donor kidney transplantation in aHUS without prophylactic eculizumab treatment appears feasible.
Keywords: Atypical hemolytic uremic syndrome (aHUS); case series; complement; drug costs; eculizumab; end-stage renal disease (ESRD); kidney transplantation; living donor; mutation; plasmapheresis; prophylactic therapy; recurrence; thrombotic microangiopathy (TMA); variant.
Copyright © 2017 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.