Abstract
IntroductionAfter a period of eculizumab shortage in Brazil, an interruption of therapy in Atypical Hemolytic Uremic Syndrome (aHUS) patients brought about high relapse rate. This study aimed to analyze the aHUS relapse rate in kidney transplant recipients who had eculizumab therapy interrupted.MethodsWe screened 800 Brazilian centers of dialysis, transplantation and nephrology throughout Brazil, through electronic mail and formal invitation by the Brazilian Society of Nephrology. We included kidney transplant recipients with aHUS who underwent interruption of eculizumab therapy for at least 30 days between January 1st, 2016 and December 31st, 2019 during the maintenance phase of treatment. Relapse was defined by the development of thrombocytopenia, hemolytic anemia, acute kidney injury or thrombotic microangiopathy in a renal graft biopsy.ResultsWe analyzed 9 patients and 9 episodes of exposure to risk of relapse. Median age was 34 (27 - 36) years, 5 (55%) patients were female, 8 (88%) had genetic study performed and 5 (55%) had any complement variant identified, among them, one (11%) had CFH variant found. Four patients (44%) had relapse after eculizumab withdrawal. Genetic characteristics related to relapses were: one patient did not have genetic study performed, one did not have pathogenic variants in complement genes identified, one had pathogenic variants found in CFI and CFB and one, in C3, CFI and CFHR1-CFHR3. The cumulative incidence of aHUS relapse at one year was 60%.ConclusionsThis study showed a high cumulative incidence of relapse (60%) at one year when eculizumab therapy in kidney transplant recipients was interrupted after a shortage in Brazil.No conflict of interest IntroductionAfter a period of eculizumab shortage in Brazil, an interruption of therapy in Atypical Hemolytic Uremic Syndrome (aHUS) patients brought about high relapse rate. This study aimed to analyze the aHUS relapse rate in kidney transplant recipients who had eculizumab therapy interrupted. After a period of eculizumab shortage in Brazil, an interruption of therapy in Atypical Hemolytic Uremic Syndrome (aHUS) patients brought about high relapse rate. This study aimed to analyze the aHUS relapse rate in kidney transplant recipients who had eculizumab therapy interrupted. MethodsWe screened 800 Brazilian centers of dialysis, transplantation and nephrology throughout Brazil, through electronic mail and formal invitation by the Brazilian Society of Nephrology. We included kidney transplant recipients with aHUS who underwent interruption of eculizumab therapy for at least 30 days between January 1st, 2016 and December 31st, 2019 during the maintenance phase of treatment. Relapse was defined by the development of thrombocytopenia, hemolytic anemia, acute kidney injury or thrombotic microangiopathy in a renal graft biopsy. We screened 800 Brazilian centers of dialysis, transplantation and nephrology throughout Brazil, through electronic mail and formal invitation by the Brazilian Society of Nephrology. We included kidney transplant recipients with aHUS who underwent interruption of eculizumab therapy for at least 30 days between January 1st, 2016 and December 31st, 2019 during the maintenance phase of treatment. Relapse was defined by the development of thrombocytopenia, hemolytic anemia, acute kidney injury or thrombotic microangiopathy in a renal graft biopsy. ResultsWe analyzed 9 patients and 9 episodes of exposure to risk of relapse. Median age was 34 (27 - 36) years, 5 (55%) patients were female, 8 (88%) had genetic study performed and 5 (55%) had any complement variant identified, among them, one (11%) had CFH variant found. Four patients (44%) had relapse after eculizumab withdrawal. Genetic characteristics related to relapses were: one patient did not have genetic study performed, one did not have pathogenic variants in complement genes identified, one had pathogenic variants found in CFI and CFB and one, in C3, CFI and CFHR1-CFHR3. The cumulative incidence of aHUS relapse at one year was 60%. We analyzed 9 patients and 9 episodes of exposure to risk of relapse. Median age was 34 (27 - 36) years, 5 (55%) patients were female, 8 (88%) had genetic study performed and 5 (55%) had any complement variant identified, among them, one (11%) had CFH variant found. Four patients (44%) had relapse after eculizumab withdrawal. Genetic characteristics related to relapses were: one patient did not have genetic study performed, one did not have pathogenic variants in complement genes identified, one had pathogenic variants found in CFI and CFB and one, in C3, CFI and CFHR1-CFHR3. The cumulative incidence of aHUS relapse at one year was 60%. ConclusionsThis study showed a high cumulative incidence of relapse (60%) at one year when eculizumab therapy in kidney transplant recipients was interrupted after a shortage in Brazil.No conflict of interest This study showed a high cumulative incidence of relapse (60%) at one year when eculizumab therapy in kidney transplant recipients was interrupted after a shortage in Brazil.
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