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Factor B Inhibition with Iptacopan in Recurrent C3 Glomerulopathy Following Kidney Transplant: A Report of Two Cases.
Escudero-Saiz, Víctor J; Gonzalez, Ángela; García-Herrera, Adriana; Larque, Ana B; Bomback, Andrew S; Morantes, Laura; Martínez-Chillarón, Marta; Ollé, Júlia; Guillén, Elena; Xipell, Marc; Molina-Andújar, Alicia; Rodríguez, Diana; Cuadrado, Elena; Cacho, Judit; Arana, Carolt; Esforzado, Núria; Bastida, Carla; Poch, Esteban; Diekman, Fritz; Cucchiari, David; Quintana, Luis F; Blasco, Miquel.
Afiliação
  • Escudero-Saiz VJ; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Gonzalez Á; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • García-Herrera A; Pathology Service, Hospital Clínic Barcelona, Barcelona, Spain.
  • Larque AB; Pathology Service, Hospital Clínic Barcelona, Barcelona, Spain.
  • Bomback AS; Division of Nephrology, Department of Medicine, Columbia University Irving Medical Center, New York, NY.
  • Morantes L; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Martínez-Chillarón M; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Ollé J; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Guillén E; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Xipell M; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Molina-Andújar A; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Rodríguez D; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Cuadrado E; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Cacho J; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Arana C; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Esforzado N; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Bastida C; Pharmacy Department, Division of Medicines, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Poch E; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Diekman F; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Cucchiari D; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Quintana LF; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
  • Blasco M; Nephrology and Kidney Transplantation Department, National Reference Center on Complex Glomerular Disease (CSUR), IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.
Kidney Med ; 6(6): 100823, 2024 Jun.
Article em En | MEDLINE | ID: mdl-38741947
ABSTRACT
C3 glomerulopathy is a rare disease caused by fluid phase dysregulation of the alternative complement pathway. Currently, treatment depends on clinical and histological severity and includes nephroprotection, unspecific immunosuppression, and terminal complement blockers (C5), without having an etiological treatment approved. C3 glomerulopathy has high recurrence rates after kidney transplantation with a high risk of graft loss. Fortunately, new molecules are being developed that specifically target the proximal alternative complement pathway, such as iptacopan, a factor B inhibitor that showed promising results in native kidneys and cases of transplant recurrence in a phase 2 clinical trial. We present 2 "real-world" cases of C3 glomerulopathy recurrence in kidney allografts treated with iptacopan, with initial excellent clinical response and safety profile, especially with early introduction. We also present follow-up biopsies that showed no C3 deposition during factor B inhibition. Our cases suggest that proximal blockade of the alternative complement pathway can be effective and safe in the treatment of C3 glomerulopathy recurrence in kidney transplantation, bringing other questions such as dual blockade (eg, in C3 and C5), the optimal patient profile to benefit from factor B inhibition or treatment duration and its potential use in other forms of membranoproliferative glomerulonephritis (eg, immune complex-mediated).
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Idioma: En Revista: Kidney Med Ano de publicação: 2024 Tipo de documento: Article País de afiliação: Espanha

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Idioma: En Revista: Kidney Med Ano de publicação: 2024 Tipo de documento: Article País de afiliação: Espanha