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Abstract: FR-PO0651

ANCA-Associated Vasculitis with Kidney Involvement at a Tertiary Care Centre: A Retrospective Case Series Characterising Disease Patterns, Histopathological Findings, and Clinical Outcomes

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Arabi, Ziad, King Abdulaziz Medical City in Riyadh, Riyadh, Saudi Arabia
  • Iqbal, Junaid, King Abdulaziz Medical City in Riyadh, Riyadh, Saudi Arabia
  • Saeed, Abdulkarim Shayif, King Abdulaziz Medical City in Riyadh, Riyadh, Riyadh Province, Saudi Arabia
  • Almanea, Abdullah Essam, King Abdulaziz Medical City in Riyadh, Riyadh, Riyadh Province, Saudi Arabia
  • Alorabi, Hussam Zaid, King Abdulaziz Medical City in Riyadh, Riyadh, Riyadh Province, Saudi Arabia
Background

ANCA-associated vasculitis (AAV) is a rare autoimmune disease causing pauci-immune necrotising glomerulonephritis and rapidly progressive kidney injury. Advanced renal disease is a major determinant of morbidity and mortality. Data from Gulf and Middle Eastern populations remain limited.

Methods

We conducted a single-centre retrospective case series of adult patients with AAV managed between January 2016 and December 2025. Thirty one patients were identified through electronic medical records. Demographic, clinical, serological, and histopathological findings were analysed. Kidney biopsies were classified using the Berden classification, and disease activity was assessed using Birmingham Vasculitis Activity Score version 3 (BVAS v3). Outcomes included end-stage renal disease (ESRD), relapse, vasculitis damage index (VDI), mortality, treatment-related adverse events, and serious infections.

Results

Out of 1,127 native kidney biopsies performed during the study period, 31 Saudi patients with biopsy-proven (AAV) were identified; 74% were female, with a median age of 53 years. Diagnoses included granulomatosis with polyangiitis (n=13), microscopic polyangiitis (n=9), eosinophilic granulomatosis with polyangiitis (n=5), and renal-limited vasculitis (n=4). MPO-ANCA positivity was present in 58%. Median eGFR at presentation was 10 mL/min, and median BVAS v3 was 14. Kidney biopsy was performed in 97%, most commonly showing mixed and crescentic Berden classes.
Induction therapy included cyclophosphamide alone (n=12), rituximab alone (n=7), and combination therapy (n=6). During follow-up, 55% progressed to ESRD, 45% required early dialysis, and complete renal recovery occurred in 26%. Mortality was 10%, and serious infections occurred in 45%. Lower baseline eGFR (p=0.003), early dialysis requirement (OR 14.4, p=0.003), and Berden class (p=0.019) predicted ESRD.

Conclusion

This study provides one of the first detailed characterisations of AAV with renal involvement from our centre and highlights severe kidney disease at presentation with poor renal outcomes. Biopsy-guided risk stratification using validated tools such as the Berden classification and BVAS/VDI scoring systems may improve prognostication and management. These findings support the need for larger prospective multicentre studies across the region.