Abstract: SA-OR024
CFHR5 Deposition in ANCA-Associated Glomerulonephritis
Session Information
- ANCA It Is
November 09, 2019 | Location: 207, Walter E. Washington Convention Center
Abstract Time: 05:06 PM - 05:18 PM
Category: Glomerular Diseases
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Brix, Silke R., Manchester University NHS Foundation Trust, Manchester Royal Infirmary, Manchester, United Kingdom
- Patel, Kajal, University of Manchester, Manchester, United Kingdom
- Busch, Martin, University Hospital Jena, Jena, Germany
- Skerka, Christine, Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany
- Stahl, Rolf A., University of Hamburg, Hamburg, Germany
- Wiech, Thorsten, Department of Pathology, University Hospital Hamburg Eppendorf, Hamburg, Germany
Background
The complement system has been found to play a role in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Complement factor H (CFH) is a negative regulator of complement C3 activation. Complement factor H related protein 5 (CFHR5) competitively binds factor 3 yet enables further complement activation. We hypothesized CFHR5 facilitates more aggressive renal ANCA disease.
Methods
Here, we investigated CFHR5 deposition in biopsies of patients with ANCA-associated glomerulonephritis (GN) from a multicenter cohort (n=207) and correlated it with clinical outcome. Granular mesangial and endothelial deposition was scored semiquantitatively (0-3).
Results
Initial renal function at time of diagnosis did not correlate with CFHR5 staining. Patients, however, who reached end stage kidney disease during-follow up were found to have a more prominent CFHR5 deposition than patients who remained dialysis independent (p<0.0001). Patients suffering from renal relapsing disease and patients who died during follow-up showed a stronger CFHR5 positivity as well (p=0.01, p=0.03).
Conclusion
Glomerular CFHR5 positivity is associated with renal outcome in ANCA-associated GN and may serve as a prognostic marker in the disease.