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Kidney Week

Abstract: TH-PO953

A Case of Familial Fibrillary Glomerulonephritis in Living Related Kidney Transplantation (LRKT)

Session Information

Category: Trainee Case Report

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Jeyabalan, Anushya, Columbia University College of Physicians and Surgeons, New York, New York, United States
  • Piras, Doloretta, Azienda Ospedaliera "G. Brotzu", Cagliari, Italy
  • Morris, Heather K., Columbia University College of Physicians and Surgeons, New York, New York, United States
  • Batal, Ibrahim, Columbia University College of Physicians and Surgeons, New York, New York, United States
  • Appel, Gerald B., Columbia University College of Physicians and Surgeons, New York, New York, United States
Introduction

Fibrillary glomerulonephritis(FGN) is a rare form of kidney disease found in ~1.0% of adult native kidney biopsies with only 4 reported cases of familial FGN. The discovery of protein DNAJ heat shock protein family (Hsp40) member B9 (DNAJB9) has aided diagnosis of FGN.

Case Description

A 49-year-old African-American man was found to have proteinuria on routine exam with UPCR 6 g/g and serum creatinine(Cr) 1.0 mg/dL. Renal biopsy later revealed FGN. Light microscopy(LM) showed diffuse mesangial matrix expansion with thickened glomerular capillary walls(GCW) (Fig 1A) with negative Congo red stain. Immunofluorescence(IF) showed 3+ staining for IgG, C3 and lambda(L), 2+ staining for kappa(K). Electron microscopy(EM) showed linear, randomly arranged fibrils with average diameter of 16 nm. DNAJB9 stain done later was strongly positive (Fig 1B). Further workup did not reveal monoclonal gammopathy and he had no known family history of kidney disease. He was treated with steroids as immunosuppressive therapy(IST). He presented to Columbia 5 years later with CKD Stage 5, thus further IST was held. At age 55, he underwent LRKT from his son. Prior to organ donation, his son had SCr of 0.88mg/dL, UA with negative blood and trace protein, UPCR 95mg/g. Post revascularization kidney biopsy showed FGN. LM showed mild mesangial proliferation with scattered double contours (Fig 2A) and negative Congo red stain. IF showed mesangial and segmental GCW staining for IgG(2+), C3(1-2+), C1(1+), K(2+) and L(2+). DNAJB9 stain was positive (Fig 2B). EM showed randomly oriented fibrils with mean diameter of 18 nm (Fig 2C). These findings are consistent with donor-derived FGN, indicating a familial form of FGN.

Discussion

We describe a form of familial FGN confirmed by DNAJB9 staining on biopsy. Presence of well-formed fibrils and glomerular changes in the donor kidney despite minimal clinical findings suggest in-depth evaluation of related donors may be important in FGN.