Rationale and objective: 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. Study design: This is a single center case series presenting our experience with KTx in aHUS. Setting and participants: 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). Results: 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. Conclusion: 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. Graphic abstract: [Figure not available: see fulltext.].

Kidney transplant in patients with atypical hemolytic uremic syndrome in the anti-C5 era: single-center experience with tailored Eculizumab / G. Ardissino, D. Cresseri, F. Tel, A. Giussani, S. Salardi, M. Sgarbanti, B. Strumbo, S. Testa, V. Capone, S. Griffini, E. Grovetti, M. Cugno, M. Belingheri, C. Tamburello, E.M. Rodrigues, M. Perrone, M. Cardillo, G. Corti, D. Consonni, L. Furian, S. Tedeschi, P. Messa, C. Beretta. - In: JN. JOURNAL OF NEPHROLOGY. - ISSN 1121-8428. - (2021 May 06). [Epub ahead of print] [10.1007/s40620-021-01045-7]

Kidney transplant in patients with atypical hemolytic uremic syndrome in the anti-C5 era: single-center experience with tailored Eculizumab

G. Ardissino
;
F. Tel;S. Salardi;B. Strumbo;V. Capone;S. Griffini;M. Cugno;P. Messa;
2021

Abstract

Rationale and objective: 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. Study design: This is a single center case series presenting our experience with KTx in aHUS. Setting and participants: 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). Results: 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. Conclusion: 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. Graphic abstract: [Figure not available: see fulltext.].
Eculizumab; Hemolytic uremic syndrome; HUS relapse; Kidney transplantation
Settore MED/09 - Medicina Interna
6-mag-2021
Article (author)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/852907
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