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Abstract: PO1737

Exploring the Role of Type I Interferons in ANCA-Associated Vasculitis

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Batten, Isabella, Centre for Medical Gerontology, School of Medicine, Trinity Translational Medicine Institute, Trinity College Dublin, Dublin, Ireland, Dublin, Ireland
  • Robinson, Mark W., Department of Biology, Human Health Institute, Maynooth University, Kildare, Ireland, Maynooth, Ireland
  • White, Arthur, School of Computer Science and Statistics, Trinity College Dublin, Dublin, Ireland, Dublin, Ireland
  • Fazekas, Barbara, Regenerative Medicine Institute, School of Medicine, National University of Ireland Galway, Galway, Ireland, Galway, Ireland
  • Walsh, Cathal D., Department of Mathematics and Statistics, University of Limerick, Limerick, Ireland
  • Wyse, Jason, School of Computer Science and Statistics, Trinity College Dublin, Dublin, Ireland, Dublin, Ireland
  • D'Arcy, Suzanne, Trinity Health Kidney Centre, Trinity Translational Medicine Institute, Trinity College Dublin, Ireland, Dublin, Ireland
  • Buettner, Antonia, Trinity Health Kidney Centre, Trinity Translational Medicine Institute, Trinity College Dublin, Ireland, Dublin, Ireland
  • Little, Mark Alan, Trinity Health Kidney Centre, Trinity Translational Medicine Institute, Trinity College Dublin, Ireland, Dublin, Ireland
  • Bourke, Nollaig M., Centre for Medical Gerontology, School of Medicine, Trinity Translational Medicine Institute, Trinity College Dublin, Dublin, Ireland, Dublin, Ireland
Background

ANCA-associated vasculitis (AAV) is a group of autoimmune diseases characterised by inflammation of small blood vessels. Type I interferons (IFNs) are cytokine mediators of the innate immune response, most known for their anti-viral properties. Dysregulation of type I IFNs is a major factor in the development of several autoimmune diseases, now termed type I interferonopathies, and thought to be the pathogenic link with chronic inflammation in these conditions. Despite evidence of type I IFNs driving autoimmunity, they have not been comprehensively studied in AAV.

We hypothesised that type I IFN responses are systemically dysregulated in AAV, indicative of a type I interferonopathy.

Methods

Matched whole blood and serum samples collected from healthy individuals (n=67), disease control patients (n=32) and AAV patients (n=71) were obtained from the Rare Kidney Disease Biobank of Ireland. qPCR was used to measure gene expression in blood of seven type I IFN stimulated genes (ISGs) characteristic of type I interferonopathies: IFI27, IFI44L, IFIT1, ISG15, RSAD2, SIGLEC1 and STAT1. Serum type I IFN regulated proteins (CXCL10, MCP-1 and CCL19) were assessed by ELISA.

Results

No significant difference in ISG gene expression was observed between control samples and AAV patients for any ISG analysed, irrespective of treatment received, age or sex. No significant differences in MCP-1, CCL19 and CXCL10 expression were observed between each cohort. CXCL10 levels were significantly lower in AAV patients on immunosuppressive treatment. Markers of Type I IFN responses did not correlate with clinical measurements of disease severity in AAV patients.

Conclusion

Systemic type I IFN responses are not dysregulated in AAV and are unlikely to contribute towards AAV pathogenesis; therefore AAV should not be considered as a type I interferonopathy

Funding

  • Private Foundation Support