Characteristics, management and outcomes of atypical haemolytic uraemic syndrome in kidney transplant patients: a retrospective national study

Author:

Portoles José12,Huerta Ana12,Arjona Emilia3,Gavela Eva24,Agüera Marisa25,Jiménez Carlos26,Cavero Teresa27,Marrero Domingo28,Rodríguez de Córdoba Santiago3,Diekmann Fritz29,Portolés José,Huerta Ana,Arjona Emilia,Gavela Eva,Luisa Maria,Jiménez Carlos,Cavero Teresa,Marrero Domingo,Redondo Dolores,Ruiz Juan Carlos,Ferrero Maria Luisa Rodríguez,Carreño Agustín,de Córdoba Santiago Rodríguez,Diekmann Fritz,

Affiliation:

1. Nephrology Department, University Hospital Puerta de Hierro, Madrid, Spain

2. RedInRen 16/009, RTYC ISCIII, Madrid, Spain

3. Center for Biological Research and CIBER of Rare Diseases, Madrid, Spain

4. Nephrology Department, University Hospital Peset, Valencia, Spain

5. Nephrology Department, University Hospital Reina Sofía, Cordoba, Spain

6. Nephrology Department, University Hospital La Paz, Madrid, Spain

7. Nephrology Department, University Hospital Doce de Octubre, Madrid, Spain

8. Nephrology Department, University Hospital Canarias, Canarias, Spain

9. Nephrology Department, University Hospital Clinic, Barcelona, Spain

Abstract

Abstract Background 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. Methods 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). Results 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. Conclusions 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.

Funder

Public Research Network

Spanish Ministerio de Economía y Competitividad-FEDER

Autonomous Region of Madrid

Publisher

Oxford University Press (OUP)

Subject

Transplantation,Nephrology

Reference27 articles.

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