Abstract: PUB148
Clinical Spectrum and Outcomes of Biopsy-Proven Anti-GBM Disease: A Single-Center Case Series
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Arabi, Ziad, King Abdulaziz Medical City in Riyadh, Riyadh, Riyadh Province, Saudi Arabia
- Alghamdi, Hazim Safar, King Abdulaziz Medical City in Riyadh, Riyadh, Riyadh Province, Saudi Arabia
- Alhejaili, Fayez F., King Abdulaziz Medical City in Riyadh, Riyadh, Riyadh Province, Saudi Arabia
- Alflaiw, Ahmad I., King Abdulaziz Medical City in Riyadh, Riyadh, Riyadh Province, Saudi Arabia
Background
Anti-glomerular basement membrane (anti-GBM) disease is a rare cause of rapidly progressive glomerulonephritis associated with significant renal morbidity. We describe the clinical features, pathology, treatment, and outcomes of biopsy-proven anti-GBM disease at our center, representing one of the largest reported series from the region.
Methods
We retrospectively reviewed 1,127 native kidney biopsies performed between 2015 and 2026. Four patients with biopsy-confirmed anti-GBM disease were identified. Clinical, serologic, histopathologic, treatment, and renal outcome data were analyzed.
Results
Four patients with biopsy-proven anti-GBM disease were identified (0.35% of biopsies). Mean age was 38 years and 75% were male. All presented with acute kidney injury and uremic symptoms; 3/4 (75%) required hemodialysis at presentation with severe renal impairment (eGFR 4–10 mL/min). Anti-GBM titers were strongly positive in 3 patients (171–195 IU/mL), while 1 patient had seronegative anti-GBM disease diagnosed only after kidney biopsy. Histopathology demonstrated crescentic glomerulonephritis.
One patient had concurrent MPO-ANCA positivity complicated by relapse with diffuse alveolar hemorrhage and progression to ESKD after 8 months. Another had seronegative anti-GBM disease with concurrent IgA nephropathy, remained dialysis-free for 4 years, then required dialysis for 2 years before successful kidney transplantation without relapse during 4 years of follow-up. All patients received corticosteroids and plasma exchange; 3 received cyclophosphamide and 1 rituximab. Overall, 2 patients remained dialysis-dependent from presentation.
Conclusion
Anti-GBM disease is a rare but aggressive cause of crescentic glomerulonephritis associated with poor renal outcomes, particularly in patients requiring dialysis at presentation. Early diagnosis and treatment are essential. Atypical presentations including ANCA double positivity and concurrent IgA nephropathy may occur. Seronegative anti-GBM disease poses a diagnostic challenge and may be missed without kidney biopsy.