Abstract: FR-PO0746
Concurrent Anti-GBM Glomerulonephritis (GN) and DNAJB9-Positive Fibrillary GN Presenting as Rapidly Progressive GN (RPGN)
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
- Dhand, Karan, Indiana University School of Medicine - Evansville Campus, Evansville, Indiana, United States
- Lee, Kenneth Ryan, Indiana University School of Medicine - Evansville Campus, Evansville, Indiana, United States
- Aeddula, Narothama Reddy, Deaconess Health System Inc, Evansville, Indiana, United States
Introduction
Concurrent anti-GBM glomerulonephritis and DNAJB9-positive fibrillary glomerulonephritis is a rare dual glomerulopathy presenting important diagnostic and therapeutic challenges.
Case Description
A 57-year-old female with a history of hashimoto’s thyroiditis, rheumatoid arthritis, and hypertension presents with gross hematuria and oliguria. On presentation, blood pressure was 213/120. Laboratory evaluation demonstrated nephrotic-range proteinuria, hematuria (>100 RBC/hpf), serum creatine 2.8 mg/dL (baseline 0.8-1 mg/dL), ESR 119 mm/hr and CRP 10.6 mg/dL. Anti-GBM antibody titers were elevated at 263 AU/mL. ANA, ANCA, MPO/PR3 antibodies, complements, hepatitis panel, HIV, and cryoglobulins were negative.
Given concerns for RPGN, pulse-dose corticosteroids were initiated empirically. Kidney biopsy demonstrated crescentic glomerulonephritis with 7/10 cellular crescents and linear IgG staining on immunofluorescence consistent with anti-GBM disease. Additional pathology revealed DNAJB9-positive fibrillary glomerulonephritis.
The patient was treated with therapeutic plasma exchange, corticosteroids, cyclophosphamide and rituximab directed towards the fibrillary component. She required approximately 24 plasma exchange sessions with improvement in anti-GBM titers from 263 at presentation to 15 AU/mL on follow-up. She did not require dialysis throughout her course and is currently being followed up and managed.
Discussion
No established guidelines exist for concurrent anti-GBM glomerulonephritis and fibrillary glomerulonephritis. Treatment must therefore, address both disease processes.
Standard anti-GBM therapy includes plasma exchange, cyclophosphamide and high-dose glucocorticoids. Plasma exchange is continued until anti-GBM titers become undetectable. Rituximab-based therapy has promising results for fibrillary glomerulonephritis
The coexistence of these two glomerular diseases may confer an especially poor renal prognosis. Renal outcomes in anti-GBM disease are strongly associated with severity at presentation, those with advanced kidney dysfunction or dialysis dependence have particularly poorer outcomes. Concurrent fibrillary GN may reduce the likelihood of renal recovery. Further reports and larger studies are needed to better characterize the optimal therapeutic approach, treatment response, and long-term treatment outcomes in patients with this rare dual glomerulopathy.