Table 2.

Summary of 15 patients receiving Eculizumab for the treatment of aHUS recurrence following renal transplantation.

Reference	Mutation	Previous transplants	Age and post-Tx course	Time from recurrence to Ecu	SCr (μmol/L) at time of Ecu	Eculizumab dosing	TMA remission achieved	Recurrence if Ecu stopped	Outcome Ecu continued SCr (μmol/L)	Follow up
Nurnberger et al. (2009)	CFH Y475S	1st Tx recurrence at 5w, PE resistant, graft loss	37y 2nd Tx, recurrence at 6w. PE resistant	5d	132	Single dose	Y	Likely (21m) graft loss	NA	NA
Chatelet et al. (2010)	C3 R570Q	1st Tx, recurrence at 5m Graft loss at 2y.	43y 2nd Tx, recurrence at 3y, PE dependent	15m	320	Ongoing	Y	NA	2 recurrences of TMAb 230	2y 5m
Legault and Boelkins (2009)	ND	No	34y 1st Tx recurrence at 1m and 5m. PE sensitive then resistant	9m	323	Ongoing	Y	NA	Remission 238	6m
Davin et al. (2010)	CFH S1191L	1st Tx, recurrence at 3d, graft loss. 2nd Tx under PE, recurrence at 10w graft loss	17y 3rd Tx, prophylactic PE. Recurrence at 4m. rescue PE intolerant at 10m.	10m	131	Ongoing	Ya	NA	Remission 130	1y 10m
Larrea et al. (2010) and Loirat and Fremeaux-Bacchi (2011)	NI	No	22y 1st Tx recurrence at 12d PE resistant	9d	415	Single dose	Y	Recurrence (11.5m) Ecu resumed	Subsequent humoral rejection Ecu stopped and graft loss	NA
Zuber et al. (2011)	CFH	1st Tx, recurrence, graft loss	24y 2nd Tx, prophylactic PI/PE recurrence 1d PE resistant	4d	500	Ongoing	Y	NA	Remission 62	9m
Al-Akash et al. (2011)	C3 R570W	1st Tx, recurrence at 4y, graft loss 2nd Tx recurrence at 2m graft loss	15y 3rd Tx, prophylactic PE, recurrence at 2m, PE partially sensitive	∼20d	202	Ongoing	Y	NA	Remission 115	1y 5m
Duran et al. (2012)	CFH Q1172X	No	32y 1st Tx,. recurrence at 1m PE sensitive further recurrence at 2m	1m	Dialysis	Ongoing	Y	NA	Remission 228	10m
Alachkar et al. (2012)	NI	1st Tx recurrence 2m, graft loss	32y 2nd Tx recurrence at 10 weeks. PE resistant	∼2w	Dialysis	8m	Y	Recurrence 5m after Ecu stopped – pneumoniac	Ecu restarted but graft loss after ATN. Ecu discontinued	NA
Ardissino et al. Zuber et al. (2012b)	CFH	No	6y 1st Tx, recurrence 2m, PE resistant	2d	442	Ongoing	Y	NA	Remission 48	25m
Zuber et al. (2012b)	CFH S1191L V1197A	1st Tx recurrence, graft loss	23 y 2nd Tx recurrence 3d. PE resistant	3d	627	Ongoing	Y	NA	Remission 65	17m
Zuber et al. (2012b)	CFH/CFHR1 hybrid	4 previous Tx – 2 due to recurrence, 2 due to thrombosis	27y 5th Tx, recurrence 3d PE partially sensitive	1m	237	Ongoing	Y	Fresh TMA lesions at 3 monthsd	Remission 204	12m
Zuber et al. (2012b)	Anti FH Ab ΔCFHR1/3	4 previous Tx, 3 due to recurrence	41y 5th Tx, recurrence 5ye PE partially sensitive	3m	89	Ongoing	Y	NA	Remission 80	9m
Guentin et al. Zuber et al. (2012b)	CFI G101R	1st Tx, recurrence graft loss	42y 2nd Tx, 15m of prophylactic PE, 8m taper recurrence at 13m after stopping. PE resistant	9w	190	Ongoing	Y	Relapse following delay prior to 5th infusion	Remission 156	4.5m
Heyne Zuber et al. (2012b)	NI	1st Tx recurrence graft loss	43y 2nd Tx, recurrence 8d no PE	1d	176	8m	Y	Relapse 3 months after stopping influenza vaccine triggered	Remission 123	14m

NI: not identified; ND: not documented; Tx: renal transplant; Ecu: Eculizumab; SCr: serum creatinine.

^aIn remission already.

^baHUS recurrence with AKI when injection delayed by 6–8 days.

^cFollowing Ecu resumption, patient had endovascular procedure leading to severe ATN and subsequent graft loss.

^dBiopsy of transplant allograft in response to falling haptoglobin level.

^eGraft biopsy due to slight decrease in renal function disclosed fresh TMA lesions.