ASN's Mission

To create a world without kidney diseases, the ASN Alliance for Kidney Health elevates care by educating and informing, driving breakthroughs and innovation, and advocating for policies that create transformative changes in kidney medicine throughout the world.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on X

Kidney Week

Abstract: FR-PO793

T Cell Hyporesponsiveness in Focal Segmental Glomerulosclerosis Responsive to Rituximab Is Associated with B Cell Activation and Production of Podocyte Damaging Factors

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: Mechanisms, including Podocyte Biology

Authors

  • Lu, Liangjian, National University Health System, Singapore, Singapore, Singapore
  • Chan, Chang-Yien, National University of Singapore, Singapore, Singapore
  • Teo, Sharon, National University Health System, Singapore, Singapore, Singapore
  • Ng, Kar Hui, National University of Singapore, Singapore, Singapore
  • Yap, Hui Kim, National University of Singapore, Singapore, Singapore
Background

The mechanistic basis of Rituximab response in idiopathic nephrotic syndrome (INS) remains uncertain. We previously showed that Rituximab-response in childhood-onset focal segmental glomerulosclerosis (FSGS) was associated with T-cell hypo-responsiveness. We aimed to investigate if dysregulated B-cell activity was associated with T-cell hypo-responsiveness prior to Rituximab.

Methods

37 patients with childhood-onset FSGS were recruited. Rituximab was administered for clinical indications, with response defined as the ability to wean off steroids and calcineurin inhibitors by 3 months after Rituximab while remaining in complete remission. T-cell hypo-responsiveness was determined by stimulated INFγ production <2.5%. B-cell activation was measured by flow cytometry of activation markers and multiplexed cytokine analysis without further stimulation. Podocytes were incubated with B-cell conditioned media, and stained for F-actin with phalloidin. Staining was scored from 0-3, with higher scores reflecting greater abnormality, i.e. more cortical F-actin and less stress fibres. Comparison between groups was performed using t-test, Wilcoxon rank-sum test or Fisher’s exact test.

Results

Baseline T-cell hypo-responsiveness was associated with Rituximab response (65% vs 29%, p=0.049). Comparing T-cell hypo-responsive and normo-responsive patients, total CD19+ B-cells were unchanged (p=0.6), but CD19+ CD80+ B-cells were increased (16% (IQR 11-26) vs 5% (IQR 5-9), p=0.006). There was a striking upregulation in resting B-cell cytokine production in patients with T-cell hypo-responsiveness, including INFγ (p= 0.005), IP10 (p=0.014) and TNFα (p=0.022). Podocytes incubated with B-cell conditioned medium from T-cell hypo-responsive patients demonstrated increased F-actin score (2.05±0.11 vs 1.36±0.15, p=0.02), but not when B-cells from T-cell normo-responsive patients were used (p=0.14). Repeat analysis 6 months post-Rituximab revealed no differences in B-cell cytokine production.

Conclusion

The association between Rituximab response and T-cell hypo-responsiveness likely reflects underlying B-cell activation, with B-cell mediated production of podocyte damaging factors. T-cell hypo-responsiveness may be an epiphenomenon reflecting T-cell exhaustion from chronic B-cell stimulation.

Funding

  • Government Support – Non-U.S.