Abstract: TH-PO0821
A Case of Fibrillary Glomerulonephritis Complicated by Cardiac Tamponade
Session Information
- Glomerular Case Reports: Membranous, PGN, GBM, and More
November 06, 2025 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Ramadhar, Julia, Rutgers Health, Newark, New Jersey, United States
- Wortman, Alexander, Rutgers Health, Newark, New Jersey, United States
- Ahmad, Madhia Bashir, Rutgers Health, Newark, New Jersey, United States
- Chenitz, Kara Beth, Veterans Health Administration Operations, East Orange, New Jersey, United States
Introduction
Fibrillary glomerulonephritis (FGN) is a rare glomerulonephritis found in 0.4-1.4% of native renal biopsies, which show randomly oriented nonbranching fibrils.1 Associations have been found with autoimmune diseases, monoclonal gammopathy, hepatitis c, and malignancies, but idiopathic FGN also exists, and is readily identified through staining for DNAJB9 protein.2,3
Case Description
Here we present a case of FGN, discovered during work-up of elevated creatinine with concurrent proteinuria. The patient missed outpatient nephrology visits for these findings and later presented to our hospital for shortness of breath, with worsened hypertension, rising creatinine, normocytic anemia and proteinuria. Further lab-work revealed nephrotic-range proteinuria, and autoimmune, rheumatologic, and basic malignant workups were negative aside from a positive hepatitis B core antibody and a transthoracic echocardiogram (TTE) showing small-moderate pericardial effusion (PEf). Renal biopsy showed fibril deposition consistent with FGN, staining strongly for DNAJB9, IgG, C3, kappa and lambda. Bone marrow biopsy for normocytic anemia was consistent with myelodysplastic syndrome (MDS). The patient was diuresed and discharged with outpatient follow-up, however presented again with recurrent shortness of breath and swelling, with TTE showing persistent PEf. Early tamponade physiology developed despite diuresis and the patient was transferred for pericardial window, with resulting exudate on studies. Upon return to our facility, he was discharged with outpatient hematology follow-up to initiate rituximab for treatment of FGN.
Discussion
The positive DNAJB9 suggests primary FGN, a rare diagnosis. We believe the exudative PEf may be secondary to FGN, as the patient was neither end-stage renal disease nor uremic at the time, both common causes of PEf in renal patients.14 Furthermore, though nephrotic syndrome may play a role in the effusion due to hypoalbuminemia, it seems just as likely that the immune-complex mediated inflammation from FGN may have caused it as well, as has been seen with lupus patients in the literature.8 Few cases in the literature demonstrate cardiac manifestations of FGN,5 with this being the sole case of pericardial effusion secondary to FGN we were able to identify, an uncommon complication of an infrequent disease process.