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Idiopathic Fibrillary Glomerulonephritis Confirmed by Immunofluorescence Staining for DNAJB9

Background: Fibrillary Glomerulonephritis (FGN) is a rare pathology that has been identified as an immune complex-mediated glomerulonephritis. While some cases of FGN are attributed to malignancy and other diseases, often they are idiopathic and must be differentiated from amyloid nephropathy. These differentiations can be confirmed by ultrastructural and light microscopy. Case Report: The patient is a 64-year-old woman presented to an outside facility with right lower quadrant pain that was worsening and associated with nausea and vomiting. She had no known medical history due to lack of primary care. She was diagnosed with acute diverticulitis with abscess leading to her transfer. Worsening renal function despite good urine output was noted during the hospitalization. Initial workup was negative for common co-morbidities associated FGN including hepatitis, human immunodeficiency virus (HIV), and malignancy. Renal biopsy confirmed FGN with endocapillary proliferation via positive staining for DNA J homolog subfamily B member 9 (DNAJB9). She was discharged home after having placement of a perm-cath and arrangement with outpatient dialysis. Discussion: Fibrillary Glomerulonephritis is a rare glomerulopathy which can manifest as nephrotic syndrome with an unclear pathogenetic mechanism. Due to the rare nature of this disease, a lack of evidence supporting a standard treatment exists. While some studies have suggested that immunomodulators such as Rituximab may be beneficial, there is not enough data to support this claim. This case highlights the importance of including FGN in a wide differential in the case of unrelenting nephrotic syndromes, even if the patient has no clear associated comorbidities.