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DNAJB9 Fibrillary Glomerulonephritis Following Rituximab-Based Therapy: A Case of Temporary Renal Recovery
Arij Ouanjine1, Fatma Fendri1, Elodie Miquelestorena-Standley2,3
1Department of Nephrology, Centre Hospitalier Universitaire d'Orléans, Orleans, FRA.
Cureus
|July 14, 2025
Summary
A patient with fibrillary glomerulonephritis experienced worsening kidney function but improved with rituximab therapy. This suggests rituximab may offer partial, time-limited control for DNAJB9-related kidney disease.
Area of Science:
- Nephrology
- Immunopathology
- Glomerular Diseases
Background:
- Fibrillary glomerulonephritis (FGN) is a rare kidney disease characterized by immune complex deposition.
- DnaJ homolog subfamily B member 9 (DNAJB9) has been identified as a key component in some FGN cases.
- Acute kidney injury and nephrotic syndrome are common presentations.
Observation:
- A 63-year-old male presented with acute kidney injury, hypertension, and nephrotic syndrome.
- Kidney biopsy confirmed glomerular DNAJB9 deposition, diagnosing FGN.
- Initial treatment with corticosteroids and rituximab led to worsening renal function and dialysis initiation.
Findings:
- A partial recovery of renal function was observed following a rituximab maintenance dose.
- The patient was able to discontinue dialysis for one year.
- This case highlights a potential partial treatment response to rituximab in DNAJB9-related FGN.
Implications:
- Rituximab-based therapy may offer a potential benefit for select patients with DNAJB9-related FGN.
- Treatment response can be partial and disease control may be time-limited.
- Further research is warranted to establish optimal therapeutic strategies for this rare condition.
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