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Kidney Week

Abstract: SA-PO0706

What to Do, Oh What to Do, to Get the Fibrillary out of You

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Hendrix, Curtis Anthony, Medical University of South Carolina, Charleston, South Carolina, United States
  • Hargous, Emily Rose, Medical University of South Carolina, Charleston, South Carolina, United States
  • Achanti, Anand, Medical University of South Carolina, Charleston, South Carolina, United States
Introduction

Fibrillary glomerulonephritis (FGN) is a rare glomerular disease with a poor prognosis due to the lack of targeted treatment options. Our case shows how a patient was able to have a reduction in proteinuria with Obintuzimab monotherapy after having multiple medication reactions and intolerances.

Case Description

The patient was initially diagnosed with FGN after a kidney biopsy, which was done due to persistent proteinuria along with the development of edema and acute kidney injury, showing 10-15nm random nonbranching filaments and glomerulus staining positive for DNAJB9. The patient started on steroids, which were ultimately discontinued after four months due to intolerance. Mycophenolate, tacrolimus, and azathioprine were all tried, but each was discontinued due to intolerance. Rituximab was started with improvement in proteinuria.

The patient moved and transferred care to our institution, with the last dose of Rituximab around one year prior. CD19/CD20 levels showed the patient had B-cell reconstitution with a urine protein to creatinine ratio (UPCR) of 535mg/g. Rituximab induction was done, but was discontinued due to the patient suffering a Grade 3 infusion reaction consisting of pruritus and hypotension requiring anti-histamines/steroids, along with discontinuing infusion. A decision was made to change therapy to Obintuzimab. Over the time it took for insurance authorization for therapy, UPCR worsened (2132 mg/g done a few weeks after 1st dose of Obintuzimab). After Obintuzimab was initiated and CD19/CD20 levels were undetectable, UPCR levels improved to <500mg/g on 2 separate samples 4 months apart.

Discussion

While on B-cell depletion therapy, the patient's creatinine has been stable, along with improved proteinuria. This case indicates the importance of B-cell depletion therapy in FGN, which normally has around a 50% chance of progression to End Stage Kidney Disease two years after diagnosis.

CD19/CD20, Proteinuria, and Creatinine Over Time
DateCD 19 Absolute #CD20 Absolute #UPCR (mg/g)Creatinine (mg/dL)
5/9/2024  5350.8
9/17/2024  7100.9
11/5/2024672  
1/31/20252.263221320.7
4/16/20250.05<259760.7
12/19/2025  2950.7
12/22/20250.01<25  
4/15/20260.00<253620.7