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Abstract: FR-PO046

An Atypical Case of DNAJB9 Negative Fibrillary Glomerulonephritis Associated With COVID-19 Infection

Session Information

Category: Coronavirus (COVID-19)

  • 000 Coronavirus (COVID-19)

Authors

  • Shehzad, Pir, Baystate Medical Center, Springfield, Massachusetts, United States
  • Khan, Sabiha M., Baystate Medical Center, Springfield, Massachusetts, United States
  • Hodgins, Spencer, Baystate Medical Center, Springfield, Massachusetts, United States
  • Freeman, Jonathan, Baystate Medical Center, Springfield, Massachusetts, United States
  • Braden, Gregory Lee, Baystate Medical Center, Springfield, Massachusetts, United States
Introduction

FGN is a rare immune-complex mediated GN characterized by the demonstration of Congo-red negative, randomly organized nonbranching fibrils with a diameter of 15 nm - 25 nm on electron microscopy and more recently the presence of DNAJB9 protein in almost all patients. FGN has been associated with HCV infection, autoimmune disease, malignancy, and monoclonal gammopathies. We report a unique case of DNAJB9 negative FGN associated with COVID-19 Infection.

Case Description

74 yo M with Afib, HTN, HFrEF, and DM presented with fatigue and dizziness. Labs revealed an elevated serum creatinine of 1.0 mg/dL above the baseline of 0.7 mg/dL. The patient was positive for COVID-19 infection without overt symptoms. The patient had been vaccinated against SARS-CoV-2. The patient developed oliguric AKI with a rapid rise in serum creatinine over 4 days. Urinalysis revealed pyuria, hematuria, and 4+ albuminuria with 14 gm of proteinuria on quantification (TP/Cr). He had hypocomplementemia, normal immunofixation, negative ANA, dsDNA, RF, ANCA, anti-GBM antibody, viral hepatitis antibodies, and cryoglobulin. MRA ruled out renal vein thrombosis. The patient was started on 1g methylprednisolone for three days. His creatinine peaked at 9.9 mg/dL and was started on hemodialysis. A kidney biopsy was done. Light microscopy showed DPGN with IF demonstrating a full house pattern most consistent with Class IV Lupus Nephritis. The Congo-red stain was negative. Electron microscopy was delayed. He was started on Euro-lupus protocol with IV Cytoxan, 500 mg IV every 2 weeks for 6 weeks, and Prednisone. The patient was discharged on maintenance Euro-lupus protocol and had an excellent response to therapy with normalization of serum creatinine to his baseline of 0.7 mg/dL and a decrease in proteinuria (TP/Cr of 0.47). The electron microscopy results later showed non-branching randomly arranged fibrils with thickness ranging from 15 nm - 30 nm characteristic of FGN. Testing for DNAJB9 was negative.

Discussion

DNAJB9 has been identified in all but a few cases of FGN. There is currently no known association between COVID-19 infection and FGN. Our case is unique since it's DNAJB9 negative and potentially establishes a new association between FGN and COVID-19 infection which can cause AKI reversible upon IV Cytoxan therapy usually reserved for crescentic FGN.