Kidney transplant in patients with atypical hemolytic uremic syndrome in the anti-C5 era: single-center experience with tailored Eculizumab.
Eculizumab
HUS relapse
Hemolytic uremic syndrome
Kidney transplantation
Journal
Journal of nephrology
ISSN: 1724-6059
Titre abrégé: J Nephrol
Pays: Italy
ID NLM: 9012268
Informations de publication
Date de publication:
Dec 2021
Dec 2021
Historique:
received:
15
01
2021
accepted:
02
04
2021
pubmed:
7
5
2021
medline:
29
1
2022
entrez:
6
5
2021
Statut:
ppublish
Résumé
Patients with atypical hemolytic uremic syndrome (aHUS) have long been considered ineligible for kidney transplantation (KTx) in several centers due to the high risk of disease recurrence, graft loss and life-threatening complications. The availability of Eculizumab (ECU) has now overcome this problem. However, the best approach towards timing, maintenance schedule, the possibility of discontinuation and patient monitoring has not yet been clearly established. This is a single center case series presenting our experience with KTx in aHUS. This study included 26 patients (16 females) with a diagnosis of aHUS, who spent a median of 5.5 years on kidney replacement therapy before undergoing KTx. We compared the aHUS relapse rate in three groups of patients who underwent KTx: patients who received no prophylaxis, patients who underwent plasma exchange, those who received Eculizumab prophylaxis. Complement factor H-related disease was by far the most frequent etiology (n = 19 patients). Untreated patients and patients undergoing pre-KTx plasma exchange prophylaxis had a relapse rate of 0.81 (CI 0.30-1.76) and 3.1 (CI 0.64-9.16) events per 10 years cumulative observation, respectively, as opposed to 0 events among patients receiving Eculizumab prophylaxis. The time between Eculizumab doses was tailored based on classic complement pathway activity (target to < 30%). Using this strategy, 12 patients are currently receiving Eculizumab every 28 days, 5 every 24-25 days, and 3 every 21 days. Our experience supports the prophylactic use of Eculizumab in patients with a previous history of aHUS undergoing KTx, especially when complement dysregulation is well documented by molecular biology.
Identifiants
pubmed: 33956337
doi: 10.1007/s40620-021-01045-7
pii: 10.1007/s40620-021-01045-7
doi:
Substances chimiques
Antibodies, Monoclonal, Humanized
0
eculizumab
A3ULP0F556
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
2027-2036Informations de copyright
© 2021. Italian Society of Nephrology.
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