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Benefit of B7-1 staining and abatacept for treatment-resistant post-transplant focal segmental glomerulosclerosis in a predominantly pediatric cohort: time for a reappraisal.
Burke, George W; Chandar, Jayanthi; Sageshima, Junichiro; Ortigosa-Goggins, Mariella; Amarapurkar, Pooja; Mitrofanova, Alla; Defreitas, Marissa J; Katsoufis, Chryso P; Seeherunvong, Wacharee; Centeno, Alexandra; Pagan, Javier; Mendez-Castaner, Lumen A; Mattiazzi, Adela D; Kupin, Warren L; Guerra, Giselle; Chen, Linda J; Morsi, Mahmoud; Figueiro, Jose M G; Vianna, Rodrigo; Abitbol, Carolyn L; Roth, David; Fornoni, Alessia; Ruiz, Phillip; Ciancio, Gaetano; Garin, Eduardo H.
Afiliación
  • Burke GW; Division of Kidney-Pancreas Transplantation, Department of Surgery, Miami Transplant Institute, University of Miami Miller School of Medicine, 1801 NW 9th Ave, Highland Professional Building, Miami, FL, 33136, USA. gburke@med.miami.edu.
  • Chandar J; Division of Pediatric Kidney Transplantation, Department of Pediatrics, Miami Transplant Institute, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Sageshima J; Division of Transplant Surgery, Department of Surgery, University of California Davis School of Medicine, Sacramento, CA, 95817, USA.
  • Ortigosa-Goggins M; Katz Family Division of Nephrology and Hypertension, Department of Medicine, and the Miami Transplant Institute, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Amarapurkar P; Division of Nephrology, Department of Medicine, Emory University School of Medicine, Atlanta, GA, 30309, USA.
  • Mitrofanova A; Research, Katz Family Division of Nephrology and Hypertension, Department of Medicine, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Defreitas MJ; Division of Pediatric Nephrology, Department of Pediatrics, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Katsoufis CP; Division of Pediatric Nephrology, Department of Pediatrics, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Seeherunvong W; Division of Pediatric Nephrology, Department of Pediatrics, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Centeno A; Transplant Clinical Pharmacy Services, Miami Transplant Institute, Jackson Memorial Hospital, Miami, FL, 33136, USA.
  • Pagan J; Katz Family Division of Nephrology and Hypertension, Department of Medicine, and the Miami Transplant Institute, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Mendez-Castaner LA; Katz Family Division of Nephrology and Hypertension, Department of Medicine, and the Miami Transplant Institute, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Mattiazzi AD; Katz Family Division of Nephrology and Hypertension, Department of Medicine, and the Miami Transplant Institute, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Kupin WL; Katz Family Division of Nephrology and Hypertension, Department of Medicine, and the Miami Transplant Institute, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Guerra G; Katz Family Division of Nephrology and Hypertension, Department of Medicine, and the Miami Transplant Institute, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Chen LJ; Division of Kidney-Pancreas Transplantation, Department of Surgery, Miami Transplant Institute, University of Miami Miller School of Medicine, 1801 NW 9th Ave, Highland Professional Building, Miami, FL, 33136, USA.
  • Morsi M; Division of Kidney-Pancreas Transplantation, Department of Surgery, Miami Transplant Institute, University of Miami Miller School of Medicine, 1801 NW 9th Ave, Highland Professional Building, Miami, FL, 33136, USA.
  • Figueiro JMG; Division of Kidney-Pancreas Transplantation, Department of Surgery, Miami Transplant Institute, University of Miami Miller School of Medicine, 1801 NW 9th Ave, Highland Professional Building, Miami, FL, 33136, USA.
  • Vianna R; Division of Kidney-Pancreas Transplantation, Department of Surgery, Miami Transplant Institute, University of Miami Miller School of Medicine, 1801 NW 9th Ave, Highland Professional Building, Miami, FL, 33136, USA.
  • Abitbol CL; Division of Liver and GI Transplantation, Department of Surgery, Miami Transplant Institute, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Roth D; Division of Pediatric Nephrology, Department of Pediatrics, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Fornoni A; Katz Family Division of Nephrology and Hypertension, Department of Medicine, and the Miami Transplant Institute, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Ruiz P; Katz Family Division of Nephrology and Hypertension, Department of Medicine, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Ciancio G; Transplant Pathology, Department of Surgery, Miami Transplant Institute, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
  • Garin EH; Division of Kidney-Pancreas Transplantation, Department of Surgery, Miami Transplant Institute, University of Miami Miller School of Medicine, 1801 NW 9th Ave, Highland Professional Building, Miami, FL, 33136, USA.
Pediatr Nephrol ; 38(1): 145-159, 2023 01.
Article en En | MEDLINE | ID: mdl-35507150
BACKGROUND: Primary FSGS manifests with nephrotic syndrome and may recur following KT. Failure to respond to conventional therapy after recurrence results in poor outcomes. Evaluation of podocyte B7-1 expression and treatment with abatacept (a B7-1 antagonist) has shown promise but remains controversial. METHODS: From 2012 to 2020, twelve patients developed post-KT FSGS with nephrotic range proteinuria, failed conventional therapy, and were treated with abatacept. Nine/twelve (< 21 years old) experienced recurrent FSGS; three adults developed de novo FSGS, occurring from immediately, up to 8 years after KT. KT biopsies were stained for B7-1. RESULTS: Nine KTRs (75%) responded to abatacept. Seven of nine KTRs were B7-1 positive and responded with improvement/resolution of proteinuria. Two patients with rFSGS without biopsies resolved proteinuria after abatacept. Pre-treatment UPCR was 27.0 ± 20.4 (median 13, range 8-56); follow-up UPCR was 0.8 ± 1.3 (median 0.2, range 0.07-3.9, p < 0.004). Two patients who were B7-1 negative on multiple KT biopsies did not respond to abatacept and lost graft function. One patient developed proteinuria while receiving belatacept, stained B7-1 positive, but did not respond to abatacept. CONCLUSIONS: Podocyte B7-1 staining in biopsies of KTRs with post-transplant FSGS identifies a subset of patients who may benefit from abatacept. A higher resolution version of the Graphical abstract is available as Supplementary information.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Glomeruloesclerosis Focal y Segmentaria / Podocitos Límite: Adult / Child / Humans Idioma: En Revista: Pediatr Nephrol Asunto de la revista: NEFROLOGIA / PEDIATRIA Año: 2023 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Alemania
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Glomeruloesclerosis Focal y Segmentaria / Podocitos Límite: Adult / Child / Humans Idioma: En Revista: Pediatr Nephrol Asunto de la revista: NEFROLOGIA / PEDIATRIA Año: 2023 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Alemania