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Kidney Week

Abstract: PO1896

Fibrillary Glomerulonephritis and Graft vs. Host Disease

Session Information

Category: Onco-Nephrology

  • 1500 Onco-Nephrology

Authors

  • Elbita, Omar Souleman Salem, University of Louisville, Louisville, Kentucky, United States
  • Garg, Gunjan, University of Louisville, Louisville, Kentucky, United States
Introduction

Fibrillary Glomerulonephritis (FGN) is rare and seen in 1% of kidney biopsies. Etiology is unkown. It is associated with malignancy, monoclonal gammopathy, autoimmune disorders and infections. There has not been FGN case reported in a pt with Graft Versus Host Disease (GVHD). We present a case AKI sec to FGN with h/o Acute Lymphocytic Leukemia, status post Allogeneic Stem Cell Transplant complicated by Gastrointestinal GVHD.

Case Description

67 yo African American female with DM, HTN, ALL s/p ASCT in remission, recently diagnosed with GI GVHD, presented with nausea, vomiting & diarrhea. Vitals: BP 140/70, Temp 37oC, HR 109. Exam showed 3+ LEs edema. Labs: Cr 3.5 mg/dL, baseline of 1.1mg/dL. Urinalysis showed hematuria and14.6g/g protein. Histology showed C3 crescentric GN. DNAJB9 stain returned positive and EM findings confirmed FGN. Patient recieved Soulmedrol, followed by Rituiximab and then Cyclophosphamide. She was dialysis dependent within 6 mos of diagnosis.

Discussion

FGN may have an undescribed association with GVHD. It is understudied because of rarity. It can present with AKI and kidney Bx is needed for diagnosis and to guide treatment. The trigger to get biopsy was the unexplained protein & blood in urine. Cr stabilized intially. However, earlier diagnosis and treatment could have delayed progression to ESRD.

Fibrils in mesangial matrix

DNAJB9 staining