Christof Aigner1, Georg A Böhmig2, Farsad Eskandary2, Harald Herkner3, Zoltán Prohászka4, Dorottya Csuka4, Renate Kain5, Martina Gaggl2, Raute Sunder-Plassmann6, Thomas Müller-Sacherer7, André Oszwald5, Gottfried Fischer8, Alice Schmidt2, Gere Sunder-Plassmann2. 1. Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Währinger Gürtel 18-20, Vienna 1090, Austria. Electronic address: christof.aigner@meduniwien.ac.at. 2. Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Währinger Gürtel 18-20, Vienna 1090, Austria. 3. Department of Emergency Medicine, Medical University Vienna, Vienna, Austria. 4. Research Laboratory, 3rd Department of Internal Medicine, and MTA-SE Research Group of Immunology and Hematology, Hungarian Academy of Sciences and Semmelweis University, Budapest, Hungary. 5. Department of Pathology, Medical University Vienna, Vienna, Austria. 6. Genetics Laboratory, Department of Laboratory Medicine, Medical University Vienna, Vienna, Austria. 7. Division of Pediatric Nephrology and Gastroenterology, Department of Pediatrics and Adolescent Medicine, Medical University Vienna, Vienna, Austria. 8. Department of Blood Group Serology and Transfusion Medicine, Medical University Vienna, Vienna, Austria.
Abstract
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) frequently leads to renal failure, and kidney transplantation bears a high risk of disease recurrence and graft loss. METHODS: Patients who received a kidney graft in our center were retrospectively identified using our Vienna Thrombotic Microangiopathy Cohort. Since 2005, the majority of aHUS patients received perioperative plasma exchange (PE) followed by plasma infusions (PI). Patients were switched to eculizumab in case of plasma intolerance or failure. Those with no preemptive therapy served as controls. We used proportional Cox regression and logistic regression to examine predictors of graft survival. RESULTS: 19 aHUS patients received 32 grafts and had a follow-up > 1 year. Eight patients received preventive plasma therapy for eight transplants and 13 patients (including 2 patients who received plasma therapy for their last transplant) had no preventive therapy for 24 grafts. The median graft survival was 2.372 days in patients, that received preemptive therapy and 411 days in patients, that did not receive preemptive treatment (hazard ratio: 0.11; p= 0.03). Four patients were switched to eculizumab because of plasma intolerance or failure. Additionally, one patient, that was not transplanted according to the above-mentioned protocol, received eculizumab for aHUS relapse. Additionally, relapse of aHUS (p = 0.01) and year of transplantation (p<0.01) had an effect on graft failure. CONCLUSIONS: This study shows that preemptive plasma therapy and eculizumab rescue in selected cases improve graft survival among kidney transplant recipients with aHUS.
BACKGROUND:Atypical hemolytic uremic syndrome (aHUS) frequently leads to renal failure, and kidney transplantation bears a high risk of disease recurrence and graft loss. METHODS:Patients who received a kidney graft in our center were retrospectively identified using our Vienna Thrombotic Microangiopathy Cohort. Since 2005, the majority of aHUSpatients received perioperative plasma exchange (PE) followed by plasma infusions (PI). Patients were switched to eculizumab in case of plasma intolerance or failure. Those with no preemptive therapy served as controls. We used proportional Cox regression and logistic regression to examine predictors of graft survival. RESULTS: 19 aHUSpatients received 32 grafts and had a follow-up > 1 year. Eight patients received preventive plasma therapy for eight transplants and 13 patients (including 2 patients who received plasma therapy for their last transplant) had no preventive therapy for 24 grafts. The median graft survival was 2.372 days in patients, that received preemptive therapy and 411 days in patients, that did not receive preemptive treatment (hazard ratio: 0.11; p= 0.03). Four patients were switched to eculizumab because of plasma intolerance or failure. Additionally, one patient, that was not transplanted according to the above-mentioned protocol, received eculizumab for aHUS relapse. Additionally, relapse of aHUS (p = 0.01) and year of transplantation (p<0.01) had an effect on graft failure. CONCLUSIONS: This study shows that preemptive plasma therapy and eculizumab rescue in selected cases improve graft survival among kidney transplant recipients with aHUS.
Authors: Heather Kerr; Andrew P Herbert; Elisavet Makou; Dariusz Abramczyk; Talat H Malik; Hannah Lomax-Browne; Yi Yang; Isabel Y Pappworth; Harriet Denton; Anna Richards; Kevin J Marchbank; Matthew C Pickering; Paul N Barlow Journal: Front Immunol Date: 2021-05-12 Impact factor: 7.561