Abstract: FR-PO0747
Fulminant Anti-GBM Disease in a Pediatric Patient with Crescentic Glomerulonephritis, Hemorrhagic Posterior Reversible Encephalopathy Syndrome (PRES), and Refractory Diffuse Alveolar Hemorrhage
Session Information
- Glomerular Diseases: ANCA Vasculitis, Anti-GBM Disease, and Crescentic GN
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Modi, Ashutosh, Baroda Medical College, Vadodara, GJ, India
- Menon, Sreelakshmi S., Baroda Medical College, Vadodara, GJ, India
- Patel, Apurva G., Nassau University Medical Center, East Meadow, New York, United States
- Patel, Kush, Columbia University Mailman School of Public Health, New York, New York, United States
Introduction
Anti-glomerular basement membrane (anti-GBM) disease is a rare cause of rapidly progressive glomerulonephritis in children. Pediatric cases are exceptionally uncommon. Simultaneous anti-GBM disease complicated by hemorrhagic PRES and diffuse alveolar hemorrhage is exceedingly rare
Case Description
A 12-year-old female with incidental sickle cell trait presented with rapidly progressive renal failure, nephrotic-range proteinuria, severe anemia, and hypertensive emergency. Creatinine 5.36 mg/dL, urine ACR 3,450 mg/g, Hb 6.3 g/dL, anti-GBM antibodies index 5.25 (ref <1.0); ANCA, ANA, ASO negative. Biopsy showed near-complete crescentic GN (16/17 glomeruli; 94%), necrotizing lesions, and linear IgG (+2) on immunofluorescence. Treatment included steroids, cyclophosphamide, plasmapheresis, and hemodialysis. Resistant hypertension required multiple agents. She developed hemorrhagic PRES with left occipital hematoma (4×4×3 cm), followed by refractory diffuse alveolar hemorrhage. Despite mechanical ventilation and maximal support, she suffered cardiopulmonary arrest and could not be resuscitated
Discussion
This case illustrates a rare fulminant multi-organ triad in pediatric anti-GBM disease: near-complete crescentic glomerulonephritis, hemorrhagic PRES, and refractory diffuse alveolar hemorrhage — all despite standard immunosuppressive therapy and plasmapheresis. Teaching points: 94% crescent burden predicts renal non-recovery and extreme mortality risk; PRES in anti-GBM RPGN may be complicated by intracranial hemorrhage altering anticoagulation and plasmapheresis decisions; alveolar hemorrhage can progress despite active antibody removal; early recognition of this fulminant trajectory guides goals-of-care discussions. Pediatric anti-GBM disease with near-complete crescent formation demands immediate multi-disciplinary intervention
20x IF microscopy showing linear IgG deposition along the glomerular basement membrane, characteristic of anti-GBM disease.