Midterm Outcomes of 12 Renal Transplant Recipients Treated With Eculizumab to Prevent Atypical Hemolytic Syndrome Recurrence.

Background: Atypical hemolytic uremic syndrome (aHUS) is an orphan disease with a high rate of recurrence after kidney transplantation. However, reports of successful prevention of posttransplant aHUS recurrence with eculizumab emerged a few years ago. To further delineate its optimal use, we describe the largest series of kidney transplant recipients treated with prophylactic eculizumab. Methods: Twelve renal transplant recipients with aHUS-related end stage renal disease received eculizumab: 10 from day 0 and 2 at the time of recurrence (days 6 and 25). Clinical and histological features, complement assessment and free eculizumab measurements were analyzed. The median follow-up was 24.6 months. Results: 5 patients had failed at least 1 previous renal transplant from aHUS. A genetic mutation was identified in 9 patients, anti-H antibodies were found in 2. No patient demonstrated biological recurrence of thrombotic microangiopathy (TMA) under treatment. Three antibody-mediated rejections (ABMRs) occurred without detectable C5 residual activity. ABMR was associated with subclinical TMA in 2 patients. One patient lost his graft after several complications, including ABMR. One patient experienced post-transplant C3 glomerulonephritis. The last median serum creatinine was 128.2 +/- 40.8 [mu]mol/l. Conclusions: These data confirm that eculizumab is highly effective in preventing posttransplantation aHUS recurrence, yet may not fully block ABMR pathogenesis. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

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