Bibliographic Details
Title: |
Benefit of B7-1 staining and abatacept for treatment-resistant post-transplant focal segmental glomerulosclerosis in a predominantly pediatric cohort: time for a reappraisal. |
Authors: |
Burke III, George W.1 gburke@med.miami.edu, Chandar, Jayanthi2, Sageshima, Junichiro3, Ortigosa-Goggins, Mariella4, Amarapurkar, Pooja5, Mitrofanova, Alla6, Defreitas, Marissa J.7, Katsoufis, Chryso P.7, Seeherunvong, Wacharee7, Centeno, Alexandra8, Pagan, Javier4, Mendez-Castaner, Lumen A.4, Mattiazzi, Adela D.4, Kupin, Warren L.4, Guerra, Giselle4, Chen, Linda J.1, Morsi, Mahmoud1, Figueiro, Jose M. G.1, Vianna, Rodrigo1,9, Abitbol, Carolyn L.7 |
Source: |
Pediatric Nephrology. Jan2023, Vol. 38 Issue 1, p145-159. 15p. 2 Color Photographs, 1 Diagram, 2 Charts, 4 Graphs. |
Subject Terms: |
*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. |
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] |
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