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Abstract: TH-PO660

DNAJB9-Associated Fibrillary Glomerulonephritis with Systemic Lupus Erythematosus: A Case Series

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials


  • Nimkar, Abhishek, Northwell Health, New Hyde Park, New York, United States
  • Hadji, Nerihan, Northwell Health, New Hyde Park, New York, United States
  • Jhaveri, Kenar D., Northwell Health, New Hyde Park, New York, United States
  • Yang, Yihe, Northwell Health, New Hyde Park, New York, United States

Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease with DNAJB9 positivity. We report 2 cases with lupus nephritis and FGN.

Case Description

Case 1: A 53-year-old woman with history of systemic lupus erythematosus (SLE), hypertension and depression presented severe proteinuria. The patient was diagnosed with SLE 20 years ago, her last lupus flare was 5 years ago. Over the course, her proteinuria worsened with spot urine protein-creatinine ratio (UPCR) was elevated at 3.54 with serum creatinine stable at 0.47mg/dl. Hep B and C, HIV, ANCA and PLA2R antibodies are negative. SLE serologies were not active. Serum C3 (148mg/dl) and C4 (42mg/dl) were in range. The serum light chain kappa/ lambda ratio was 1.05. She was on MMF and hydroxychloroquine. A kidney biopsy confirmed lupus nephritis membranous type/ class V and glomerular dense fibrillary deposits suggestive of fibrillary glomerulonephritis. DNAJB9 antibody staining was positive. Patient received rituximab infusion therapy (1gm X 2 doses). Two months of follow up showed improvement in UPCR (0.9) with stable serum creatinine at 0.41mg/dl. One year out, the patient remains in remission.
Case 2: A 65-year-old female with SLE (diagnosed ~40 years ago), hypertension and arthritis who presented with rising serum creatinine at 2.4 mg/dl. Her UPCR was stable at 0.3. Serological work up was negative. Serum C3 complement levels were mildly low (74mg/dl) and serum C4 complement levels were in range (18mg/dl). The Serum light chain kappa/ lambda ratio was 1.86. The patient was on MMF. Her BP was elevated, and lower extremity edema was present on exam. Kidney biopsy showed fibrillary glomerulonephritis with mesangioproliferative pattern and abundant electron dense material with fibrillary substructure infiltrating mesangium and glomerular basement membranes. mmunohistochemical staining of DNAJB9 was positive in glomeruli. Patient received rituximab infusion therapy. 9 months follow up showed stable UPCR (0.2) with elevated but stable serum creatinine at 1.86mg/dl. Her BP improved without any medications and clinically lower extremity edema disappeared. There was no progression of disease.


The association between SLE and DNAJBP associated FGN is unclear. Rituximab may serve as a potential treatment strategy.