*Purpose: Atypical hemolytic uremic syndrome (aHUS) is caused by genetic mutations of components of the alternative complement pathway in 60-70% of cases; and has a high recurrence rate after kidney transplantation. Eculizumab, an antibody inhibiting complement factor 5a, has been used in prevention and treatment of post-transplant aHUS. We intend to report our center’s 20-year experience with management of aHUS in kidney transplant recipients (KTR), highlighting the use of Eculizumab in the prevention and treatment of aHUS recurrence.

*Methods: We performed a retrospective analysis of KTR between 1999 and 2018. We reviewed clinical data, including genetic testing for complement factor mutations, post-transplant course, and response to therapy including therapeutic and prophylactic use of eculizumab.

*Results: 18 patients with aHUS underwent 35 kidney transplants during the study period. Median age at time of last transplant was 37 (range 27-59) years. In the cohort, 72% were female, 78% Caucasian, and 61% had live donor transplant as the last transplant. 58% had at least one previous kidney transplant that failed due to aHUS. 58% had genetic mutation of the alternative complement pathway: 37% were noted to have complement factor H (CFH) mutations, 21% had other complement factor mutations or CFH related gene deletions and 42% did not have any complement mutation identified on genetic testing. Median duration of follow up was 46 (range 6-237) months. 56% (10/18) received Eculizumab prophylaxis at the time of transplant. Eculizumab was discontinued in 3 patients at 6 months post-transplant, where no genetic mutation was identified. No aHUS recurrence occurred during the follow up. Of the remaining 8 patients not treated with prophylactic Eculizumab, 56% (5/9) developed recurrent aHUS in the kidney allograft. 60% (3/5) were treated with Eculizumab with two patients progressed rapidly to ESRD. Median duration of Eculizumab therapy was 13 (range 1-76) months. At the end of study period, 54% of patients who received Eculizumab continue to be on treatment. No meningococcal infections occurred during the follow up.

*Conclusions: Our data supports the effectiveness of Eculizumab in preventing recurrence of aHUS after kidney transplantation. Recurrence is common in patients with CFH mutations who are not treated with prophylactic Eculizumab. Treatment discontinuation is possible in low genetic risk patients. Long term Eculizumab has been safe; with no major infectious complications.

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