학술논문
Benefit of B7-1 staining and abatacept for treatment-resistant post-transplant focal segmental glomerulosclerosis in a predominantly pediatric cohort: time for a reappraisal.
Document Type
Article
Author
Burke III, 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.
Source
Subject
*STAINS & staining (Microscopy)
*BIOPSY
*NEPHROTIC syndrome
*KIDNEY transplantation
*PEDIATRICS
*PATIENTS
*SURGERY
*DISEASE relapse
*COMPARATIVE studies
*PROTEINURIA
*DESCRIPTIVE statistics
*EPITHELIAL cells
*FOCAL segmental glomerulosclerosis
*ABATACEPT
*LONGITUDINAL method
*TRANSPLANTATION of organs, tissues, etc.
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Language
ISSN
0931-041X
Abstract
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 [ABSTRACT FROM AUTHOR]