RT Journal Article
T1 Characteristics, management and outcomes of atypical haemolytic uraemic syndrome in kidney transplant patients: a retrospective national study.
A1 Portoles, José
A1 Huerta, Ana
A1 Arjona, Emilia
A1 Gavela, Eva
A1 Agüera, Marisa
A1 Jiménez, Carlos
A1 Cavero, Teresa
A1 Marrero, Domingo
A1 Rodríguez de Córdoba, Santiago
A1 Diekmann, Fritz
A1 Matrix Investigators, 
K1 aHUS atypical haemolytic uraemic syndrome
K1 aHUS de novo
K1 eculizumab
K1 genetic study
K1 kidney transplantation recurrence
AB Kidney transplantation (KTx) is a strong trigger for the development of either recurrent or de novo atypical haemolytic uraemic syndrome (aHUS). According to previous studies, eculizumab (ECU) is effective for prophylaxis and for treatment of recurrence. We evaluated the experiences of Spanish patients with recurrent and de novo aHUS associated with KTx, treated or not treated with ECU. In the de novo group, we classified patients as having early de novo (during the first month) or late de novo aHUS (subsequent onset). We analysed 36 cases of aHUS associated with KTx. All of the 14 patients with pre-KTx diagnosis of aHUS were considered to have high or moderate risk of recurrence. Despite receiving grafts from suboptimal donors, prophylactic ECU was effective for avoiding recurrence. The drug was stopped only in two cases with low-moderate risk of recurrence and was maintained in high-risk patients with no single relapse. There were 22 de novo aHUS cases and 16 belonged to the early de novo group. The median time of onset in the late group was 3.4 years. The early group had a better response to ECU than the late group, probably due to earlier diagnosis and use of the drug. No genetic pathogenic variant was detected in de novo aHUS cases, suggesting a secondary profile of the disease. ECU was stopped in all de novo patients with no relapses. ECU was well tolerated in all cases. Both groups (pre-aHUS and de novo) presented different clinical profiles, management approaches and outcomes. One should consider aHUS regardless of time after KTx. Genetic studies are crucial to stratify risks of relapse and to determine necessary lengths of treatment. We suggest short ECU treatment for de novo cases without pathogenic mutation and that ECU treatment be considered pre-emptively for patients with moderate or high risk of recurrence.
SN 2048-8505
YR 2020
FD 2020-08-13
LK https://hdl.handle.net/10668/25754
UL https://hdl.handle.net/10668/25754
LA en
DS RISalud
RD Apr 7, 2025