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Abstract: TH-PO691

The Epidemiology of ANCA-Associated Vasculitis over Two Decades at a Large Tertiary Centre

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Storrar, Joshua, Northern Care Alliance NHS Foundation Trust, Salford, Manchester, United Kingdom
  • Raza, Sayyid M Ammar, Northern Care Alliance NHS Foundation Trust, Salford, Manchester, United Kingdom
  • Chinnadurai, Rajkumar, Northern Care Alliance NHS Foundation Trust, Salford, Manchester, United Kingdom
  • Sinha, Smeeta, Northern Care Alliance NHS Foundation Trust, Salford, Manchester, United Kingdom
  • Kalra, Philip A., Northern Care Alliance NHS Foundation Trust, Salford, Manchester, United Kingdom
Background

ANCA-associated vasculitis (AAV) is a systemic condition affecting small blood vessels and commonly causes kidney disease. It has a reported incidence of around 20-25 per million per year. Here we aimed to describe its epidemiology at our renal centre over a 23-year period and to compare granulmatosis with polyangiitis (GPA) and miroscopic polyangiitis (MPA).

Methods

We identified all patients with AAV between January 2000 and December 2022 through our biopsy database and electronic patient record (EPR). The cohort after exclusions totalled 278. We collected baseline demographic data, baseline laboratory values, treatment received and outcomes including progression to renal replacement therapy (RRT), relapse rates and mortality.

Results

Median age of the cohort was 63.5 years, 52.5% were male and 92.4% White. GPA was seen in 49.3% and MPA in 42.1%. Median eGFR at presentation was 18mls/min/1.73m2. RRT at presentation occurred in 19.4%. Relapse occurred in 23%, progression to RRT in 26.6% and 1-year mortality was 3.2%. Median follow up duration was 54 months. Those with GPA were younger, had higher incidence of ENT disease at presentation, were more likely to relapse and had greater 1- and 5- year survival. Mortality rate per hundred person years improved from 2010 onwards.

Conclusion

This is one of the largest retrospective observational studies conducted on AAV, which reports real world epidemiology of this rare condition. AAV presents with advanced renal disease and there remain important differences in presentation and outcomes between GPA and MPA.

The Salford glomerulonephritis research group was supported by an unrestricted project grant from CSL Vifor.

Demographics, laboratory results and major outcomes for the total cohort, those with GPA and those with MPA.

Funding

  • Commercial Support – CSL Vifor