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Abstract: TH-PO0711

Endothelial Injury Is Linked to Silent Inflammation in Idiopathic Nephrotic Syndrome in Children

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Islam, Md Imtiazul, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Fetsko, Audrey Rose, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Phillips, Melonie Anne, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Kallash, Mahmoud, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Wang, Xin, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Dougherty, Julie, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Smoyer, William E., Nationwide Children's Hospital, Columbus, Ohio, United States
  • Johnson, Richard J., University of Colorado Anschutz Medical Campus, Aurora, Colorado, United States
  • Cara-Fuentes, Gabriel M., Nationwide Children's Hospital, Columbus, Ohio, United States
Background

Idiopathic Nephrotic Syndrome (INS) is considered an auto-immune, non-inflammatory, podocytopathy. Steroids, gold standard treatment in INS, are thought to exclusively act on immune cells and podocytes. Recent data showed evidence of endothelial injury, but its significance is unknow. Here, we tested the hypothesis that endothelial injury is linked with silent inflammation and that steroids exert an anti-inflammatory effect on glomerular endothelial cells.

Methods

We studied 150 children with INS. In serum, we measured angiopoietin-2 (Ang2) and TNFR1 and TNFR2, as surrogate markers of endothelial injury and inflammation, respectively, by ELISA. Samples were measured during relapse or remission, including serial samples in a subset of patients. We examined expression of the above target in human kidney by accessing available scRNA seq. Human glomerular endothelial cells (GEnC) were incubated with serum from healthy controls and INS patients in relapse with or without the addition of dexamethasone to culture media, and western blots of markers of inflammation (eNOS, GCR, ANG2) were performed.

Results

Ang-2 was higher in ~60% and ~40% of children with active INS or in remission, respectively, than in controls. TNFR1 and TNFR2 levels were increased in ~90% and 50%, respectively, of children with active INS or remission (Fig 1). All markers decreased by ~40% following steroids but remained high in some patients in remission. There was a strong correlation between TNFR1/TNFR 2 and Ang2. Patients with high Ang2, TNFR1 and TNFR2 showed a trend toward longer time to achieve remission. By scRNA seq of kidney tissue, Ang-2 and TNFR2 expression was increased in INS, predominantly in endothelial cells. In cell culture, INS sera decreased Ang2 expression compared to control, and this was mitigated by Dexamethasone (Fig 2).

Conclusion

Endothelial injury is linked to silent inflammation in INS. GEnC are a plausible cellular and therapeutic target in these patients.

Digital Object Identifier (DOI)