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Abstract: TH-PO509

Prognosis of Diffuse Crescentic Fibrillary Glomerulonephritis

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials

Authors

  • Javaugue, Vincent, Mayo Clinic Minnesota, Rochester, United States
  • Said, Samar M., Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Bu, Lihong, Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Bridoux, Frank, Centre Hospitalier Universitaire de Poitiers, Poitiers, France
  • Fervenza, Fernando C., Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Leung, Nelson, Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Nasr, Samih H., Mayo Clinic Minnesota, Rochester, Minnesota, United States
Background

Diffuse crescentic involvement is very rare in fibrillary glomerulonephritis (FGN). Little is known about the prognosis and the therapeutic management of these patients.

Methods

We retrospectively reviewed the pathology archives at Mayo Clinic and University Hospital of Poitiers from 1997-2021 for FGN with crescents involving ≥50% of glomeruli.

Results

Twenty-two patients (50% female, 91% Caucasian, median age 59 years) were included (incidence of 2.7% in biopsies of FGN). All patients presented with rapidly progressive glomerulonephritis (RPGN) and 9 required dialysis at diagnosis. Eight patients had CKD stage ≥3. Associated conditions included autoimmune disease in 6, malignancy in 3 and hepatitis C in 2 cases. Four had positive ANCA and 1 had an anti-GBM antibody. By light microscopy, the median % of glomeruli with crescents was 64%. Glomeruli showed strong staining for DNAJB9 in 11 tested cases. By immunofluorescence, all cases showed glomerular positivity for IgG (polytypic in 19/22) with IgG4 restriction in most cases, and linear GBM staining mimicking anti-GBM nephritis in 6. Nineteen patients received immunosuppressive therapy (steroids alone, n=3; or with cyclophosphamide, n=11; rituximab, n=4; rituximab + cyclophosphamide, n=1) associated with plasmapheresis in 4 cases. Kaplan-Meier analysis showed poor renal outcome with a median renal survival of 3 weeks (Figure). Three patients received kidney transplantation without evidence of recurrence 18-, 48- and 56-months post transplantation, respectively.

Conclusion

Our study highlights a dismal kidney prognosis of crescentic FGN regardless of the type of therapy and suggests that kidney transplant is probably the best option for fit patients with RPGN secondary to diffuse crescentic FGN.

Kaplan-Meier renal survival analysis.