Abstract: SA-PO0741
Crescentic Fibrillary Glomerulonephritis with Severe AKI: Renal Recovery After Rituximab Induction
Session Information
- Glomerular Diseases: Complement-Mediated Glomerulopathies and Infection-Related GN
October 24, 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
- Renfrow, Amanda Joy Cox, WVU Medicine, Morgantown, West Virginia, United States
- Khan, Salwa, WVU Medicine, Morgantown, West Virginia, United States
- Murari, Ujjwala, WVU Medicine, Morgantown, West Virginia, United States
- Kaushal, Amit, WVU Medicine, Morgantown, West Virginia, United States
- Schmidt, Rebecca J., WVU Medicine, Morgantown, West Virginia, United States
Introduction
Fibrillary glomerulonephritis (FGN) is an uncommon DNAJB9-positive glomerular disease with no standardized treatment. Crescentic FGN is a rare, severe subtype with poor prognosis. Rituximab has emerging evidence in FGN but data in crescentic disease are limited. We describe a woman with crescentic FGN and dialysis-requiring AKI who recovered renal function with rituximab induction.
Case Description
A 58-year-old woman with CKD3b (baseline creatinine 1.6-1.7 mg/dL), type 2 diabetes, and gastric bypass developed AKI and nephrotic-range proteinuria after admission for acute cholecystitis. Creatinine peaked at 7.9 mg/dL, UPCR was 9,269 mg/g, and albumin nadired at 1.1 g/dL. SPEP/UPEP, sFLC ratio, ANA, anti-dsDNA, anti-GBM, ANCA, PLA2R, cryoglobulin, hepatitis B/C, and HIV were negative. Kidney biopsy showed fibrillary GN with fibrinoid necrosis and cellular crescents in 2 of 16 non-sclerotic glomeruli and moderate IFTA. DNAJB9 immunostain was positive, confirming the diagnosis. She received intravenous methylprednisolone 500 mg daily for three days, oral prednisone taper, and rituximab 1 g induction (three weekly doses). She required intermittent hemodialysis during the acute phase. After induction, creatinine improved and dialysis was discontinued. The course was complicated by steroid-induced psychosis prompting readmission and a transient AKI relapse managed with brief reinitiation of dialysis and steroid taper acceleration.
Discussion
Crescentic FGN is the most aggressive form of an already uncommon disease and tends to progress to kidney failure within months. There is no agreed treatment; published cases describe corticosteroids, cyclophosphamide, and rituximab in various combinations. Our patient came off dialysis after rituximab induction, which is consistent with the growing case-series evidence that B-cell depletion can change the trajectory in FGN. The diagnosis itself rested on DNAJB9 positivity once monoclonal, infectious, and autoimmune causes had been excluded. A separate issue this case raises is what happens after the acute treatment phase. Pulse-dose and high-dose oral steroids were necessary up front, but the patient was readmitted with steroid-induced psychosis, and the AKI briefly returned. Tapering steroids more quickly once rituximab has had time to act might have spared her the readmission.