Myeloperoxidase immunohistochemical staining can identify glomerular endothelial cell injury in dense deposit disease

Background Previous studies have demonstrated residual complement-mediated deposits in repeat kidney biopsies of C3 glomerulopathies (C3G) (dense deposit disease (DDD) and C3 glomerulonephritis) following eculizumab treatment, despite some clinical improvement. With residual complement deposition, i...

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Bibliographic Details
Published in:Pediatric nephrology (Berlin, West) West), 2021-12, Vol.36 (12), p.4003-4007
Main Authors: Blatt, Neal B., Kumar, Tripti, Wickman, Larysa T., Kanaan, Hassan D., Chang, Anthony, Zhang, Ping L.
Format: Article
Language:English
Subjects:
Biopsy
Brief Report
Care and treatment
Cell injury
Child, Preschool
Complement activation
Complement component C3
Complement system
Demographic aspects
Diagnosis
Diagnostic immunohistochemistry
Endothelial cells
Endothelial Cells - pathology
Endothelium
Glomerulonephritis
Glomerulonephritis - diagnosis
Glomerulonephritis - drug therapy
Glomerulonephritis, Membranoproliferative - diagnosis
Humans
Immunohistochemistry
Inhibitor drugs
Kidney diseases
Male
Medicine
Medicine & Public Health
Methods
Monoclonal antibodies
Nephrology
Pediatrics
Peroxidase
Staining and Labeling
Urology
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Summary:Background Previous studies have demonstrated residual complement-mediated deposits in repeat kidney biopsies of C3 glomerulopathies (C3G) (dense deposit disease (DDD) and C3 glomerulonephritis) following eculizumab treatment, despite some clinical improvement. With residual complement deposition, it is difficult to determine whether there is a reduced complement-mediated endothelial cell injury. We validated that myeloperoxidase (MPO) immunohistochemical staining identified glomerular endothelial cell injury in crescentic glomerulonephritis and C3G. Case (diagnosis/treatment) We report that MPO staining in the glomerular endothelium of the post-treatment kidney biopsy was significantly reduced after 3 years of eculizumab treatment and clinical improvement in a 5-year-old boy with initial DDD and secondary crescent formation. Conclusion We find that immunostaining for MPO is a useful method to compare glomerular endothelial injury in C3G following eculizumab treatment. This finding also supports the notion that eculizumab, a C5 blocker, may not mainly block C3 deposits in the glomeruli but significantly blocks final activation of the complement cascade, thus reducing glomerular endothelial cell injury.
ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-021-05262-x