title: Eculizumab for atypical hemolytic uremic syndrome recurrence in renal transplantation.
abstract: Eculizumab (anti-C5) has been sporadically reported as an efficient therapy for atypical hemolytic uremic syndrome (aHUS). However, the lack of series precludes any firm conclusion about the optimal use of anti-C5 for preventing or treating aHUS posttransplant aHUS recurrence. We thoroughly studied 22 renal transplant recipients with aHUS who received off-label therapy with anti-C5, including 12 cases, which have not been reported yet. Nine patients, all carrying a complement genetic abnormality associated with a high risk of aHUS recurrence, received prophylactic anti-C5 therapy to prevent posttransplant recurrence. Eight of them had a successful recurrence-free posttransplant course and achieved a satisfactory graft function, while the remaining patient experienced early arterial thrombosis of the graft. Thirteen renal transplant recipients were given anti-C5 for posttransplant aHUS recurrence. A complete reversal of aHUS activity was obtained in all of them. Importantly, the delay of anti-C5 initiation after the onset of the aHUS episode inversely correlated with the degree of renal function improvement. Three patients in whom anti-C5 was subsequently stopped experienced a relapse. Altogether these data suggest that long-term eculizumab is highly effective for preventing and treating posttransplant aHUS recurrence. Our study also indicates that anti-C5 should be promptly started if a recurrence occurs.
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Hôpital Necker; Université Paris Descartes, Sorbonne Paris Cité; Department of Renal Transplantation, Paris, France. julien.zuber@nck.aphp.fr
authors.forename: J
authors.initials: J
authors.lastname: Zuber
authors.forename: M
authors.initials: M
authors.lastname: Le Quintrec
authors.forename: S
authors.initials: S
authors.lastname: Krid
authors.forename: C
authors.initials: C
authors.lastname: Bertoye
authors.forename: V
authors.initials: V
authors.lastname: Gueutin
authors.forename: A
authors.initials: A
authors.lastname: Lahoche
authors.forename: N
authors.initials: N
authors.lastname: Heyne
authors.forename: G
authors.initials: G
authors.lastname: Ardissino
authors.forename: V
authors.initials: V
authors.lastname: Chatelet
authors.forename: L-H
authors.initials: LH
authors.lastname: Noël
authors.forename: M
authors.initials: M
authors.lastname: Hourmant
authors.forename: P
authors.initials: P
authors.lastname: Niaudet
authors.forename: V
authors.initials: V
authors.lastname: Frémeaux-Bacchi
authors.forename: E
authors.initials: E
authors.lastname: Rondeau
authors.forename: C
authors.initials: C
authors.lastname: Legendre
authors.forename: C
authors.initials: C
authors.lastname: Loirat
mesh_terms: D000293:Adolescent; D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome; D002648:Child; D002675:Child, Preschool; D003182:Complement C5 / Q000037:antagonists & inhibitors / Q000276:immunology; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D007223:Infant; D016030:Kidney Transplantation / Q000009:adverse effects*; D008297:Male; D011183:Postoperative Complications; D011379:Prognosis; D012189:Retrospective Studies; D055502:Secondary Prevention; D055815:Young Adult
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D003182:Complement C5; C481642:eculizumab