TY - JOUR
T1 - Eculizumab for atypical hemolytic uremic syndrome recurrence in renal transplantation
AU - Zuber, J.
AU - Le Quintrec, M.
AU - Krid, S.
AU - Bertoye, C.
AU - Gueutin, V.
AU - Lahoche, A.
AU - Heyne, N.
AU - Ardissino, G.
AU - Chatelet, V.
AU - Noël, L. H.
AU - Hourmant, M.
AU - Niaudet, P.
AU - Frémeaux-Bacchi, V.
AU - Rondeau, E.
AU - Legendre, C.
AU - Loirat, C.
PY - 2012/12
Y1 - 2012/12
N2 - 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.
AB - 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.
KW - Atypical hemolytic uremic syndrome
KW - eculizumab
KW - renal transplant recipient
UR - http://www.scopus.com/inward/record.url?scp=84870534251&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84870534251&partnerID=8YFLogxK
U2 - 10.1111/j.1600-6143.2012.04252.x
DO - 10.1111/j.1600-6143.2012.04252.x
M3 - Article
C2 - 22958221
AN - SCOPUS:84870534251
VL - 12
SP - 3337
EP - 3354
JO - American Journal of Transplantation
JF - American Journal of Transplantation
SN - 1600-6135
IS - 12
ER -