Abstract: FR-PO0798
Dual Phospholipase A2 Receptor (PLA2R)/NELL-1 Membranous Nephropathy with p-ANCA Positivity Presenting with Seizures and Respiratory Failure
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
- Anshul, Fnu, Southeast Health, Dothan, Alabama, United States
- Naveed, Osama Kunwer, Southeast Health, Dothan, Alabama, United States
- Aparna, Fnu, Piedmont Columbus Regional - Midtown, Columbus, Georgia, United States
Introduction
Dual PLA2R and NELL1 positivity in membranous nephropathy is rare, and its clinical significance remains unclear.
Case Description
A 31-year-old woman presented with new-onset seizures after several days of nausea, vomiting, and diarrhea, with hypomagnesemia and hyponatremia. Brain MRI showed bilateral hippocampal FLAIR hyperintensities without enhancing lesions. Seizures were considered multifactorial in the setting of electrolyte derangements and possible autoimmune contribution. During hospitalization, she developed acute hypoxemic respiratory failure requiring invasive mechanical ventilation for hypoxemia. Chest CT demonstrated multifocal ground-glass opacities, and bronchoscopy raised concern for possible alveolar hemorrhage. Vasculitis evaluation revealed pANCA positivity, but cerebral angiography, renal function, and additional serologic workup were unrevealing, and there was no evidence of ANCA-associated vasculitis. She improved markedly with corticosteroids and was successfully extubated. Subsequent examination revealed edema, and urine studies showed nephrotic-range proteinuria. Serum PLA2R antibodies were positive, and kidney biopsy confirmed membranous nephropathy with dual PLA2R and NELL1 positivity.
Discussion
This case highlights the diagnostic complexity of concurrent neurologic, pulmonary, and renal findings in a patient with multiple autoantibodies. Dual PLA2R/NELL1 membranous nephropathy is uncommon, and pANCA positivity may confound evaluation for vasculitis when clinicopathologic findings are discordant. This constellation raises the possibility that dual antigen positivity reflects a broader systemic immune dysregulation rather than kidney-limited disease. Careful integration of clinical, serologic, and histopathologic data is essential to avoid misclassification and guide management.