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Eculizumab interruption in atypical hemolytic uremic syndrome due to shortage: analysis of a Brazilian cohort.
Neto, Miguel Ernandes; de Moraes Soler, Lucas; Vasconcelos, Halita Vieira Gallindo; Nga, Hong Si; Bravin, Ariane Moyses; Borges, Julio Cesar Andriotti; Gonçalves, Rodrigo Costa; Von Kriiger, Rodrigo Brum; Quinino, Raquel Martins; de Mello Santana, Viviane Brandão Bandeira; de Holanda, Maria Izabel; Vaisbich, Maria Helena; Naseri, Alice Pignaton; Kirsztajn, Gianna Mastroianni; Palma, Lilian Monteiro Pereira; Andrade, Luís Gustavo Modelli.
Afiliação
  • Neto ME; Department of Internal Medicine, São Paulo State University (UNESP), Botucatu, SP, Brazil. m_ernandes@yahoo.com.br.
  • de Moraes Soler L; Hospital BP-a Beneficência Portuguesa de São Paulo, São Paulo, SP, Brazil. m_ernandes@yahoo.com.br.
  • Vasconcelos HVG; Department of Internal Medicine, São Paulo State University (UNESP), Botucatu, SP, Brazil.
  • Nga HS; Departamento de Nefrologia da Santa Casa de São Carlos, São Carlos, SP, Brazil.
  • Bravin AM; Department of Internal Medicine, São Paulo State University (UNESP), Botucatu, SP, Brazil.
  • Borges JCA; Department of Internal Medicine, São Paulo State University (UNESP), Botucatu, SP, Brazil.
  • Gonçalves RC; Fresenius Medical Care-Unidade Perdizes, São Paulo, SP, Brazil.
  • Von Kriiger RB; Hospital de Urgências Governador Otávio Lage de Siqueira (HUGOL), Goiânia, GO, Brazil.
  • Quinino RM; Hospital de Urgências Governador Otávio Lage de Siqueira (HUGOL), Goiânia, GO, Brazil.
  • de Mello Santana VBB; Hospital Universitário Onofre Lopes, Universidade Federal do Rio Grande do Norte, Natal, RN, Brazil.
  • de Holanda MI; Instituto Hospital de Base, Brasília, DF, Brazil.
  • Vaisbich MH; Hospital Federal de Bonsucesso-Serviço de Nefrologia e Transplante, Rio de Janeiro, RJ, Brazil.
  • Naseri AP; Instituto da Criança-University of São Paulo, São Paulo, SP, Brazil.
  • Kirsztajn GM; Setor de Nefrologia da Unidade de Gestão de Transplantes da Universidade Federal do Espírito Santo, Vitória, ES, Brazil.
  • Palma LMP; Departamento de Medicina (Nefrologia) da Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil.
  • Andrade LGM; Nefrologia Pediátrica-Departamento de Pediatria da Universidade Estadual de Campinas (UNICAMP), Campinas, SP, Brazil.
J Nephrol ; 34(4): 1373-1380, 2021 Aug.
Article em En | MEDLINE | ID: mdl-33387344
BACKGROUND: The risk of eculizumab therapy discontinuation in patients with atypical hemolytic uremic syndrome (aHUS) is unclear. The main objective of this study was to analyze the risk of aHUS relapse after eculizumab interruption due to drug shortage in Brazil. METHODS: We screened all the registered dialysis centers in Brazil (n = 800), willing to participate in the aHUS Brazilian shortage cohort, through electronic mail and formal invitation by the Brazilian Society of Nephrology. We included patients with aHUS whose eculizumab therapy underwent unplanned discontinuation 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 (TMA) in a kidney biopsy. RESULTS: We analyzed 25 episodes of exposure to risk of relapse, from 24 patients. Median age was 33 (6-53) years, 18 (72%) were female, 9 (36%) had a functioning renal graft, 5 (20%) were undergoing dialysis. CFH variant was found in 8 (32%) episodes. There were 11 relapses. The risk of relapse was 34%, 44.5% and 58% at 114, 150 and 397 days, respectively. No baseline variable was related to relapse in Cox multivariate analysis, including CFH variant. CONCLUSIONS: In this study, the cumulative incidence of aHUS relapse at 397 days was 58% after eculizumab interruption. The presence of complement variant does not seem to be associated with a higher relapse rate. The eculizumab interruption was deemed not safe, considering that the rate of relapse was high.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Microangiopatias Trombóticas / Síndrome Hemolítico-Urêmica Atípica Tipo de estudo: Risk_factors_studies Limite: Adult / Female / Humans País/Região como assunto: America do sul / Brasil Idioma: En Revista: J Nephrol Assunto da revista: NEFROLOGIA Ano de publicação: 2021 Tipo de documento: Article País de afiliação: Brasil País de publicação: Itália
Texto completo
Adicionar na Minha BVS
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Microangiopatias Trombóticas / Síndrome Hemolítico-Urêmica Atípica Tipo de estudo: Risk_factors_studies Limite: Adult / Female / Humans País/Região como assunto: America do sul / Brasil Idioma: En Revista: J Nephrol Assunto da revista: NEFROLOGIA Ano de publicação: 2021 Tipo de documento: Article País de afiliação: Brasil País de publicação: Itália