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Fibrillary glomerulonephritis: an observational study of clinical-pathological features and outcomes in patients from

Jean Patrick1, Madeline Charles-Rudwick1, Charlotte Quinn2

  • 1Department of Renal Medicine, James Paget University Hospitals NHS Foundation Trust, Great Yarmouth, UK.

Clinical Kidney Journal
|July 2, 2025
PubMed
Abstract

Insights

Fibrillary glomerulonephritis (FGN) has poor renal outcomes. Female sex, proteinuria, and diffuse proliferative glomerulonephritis (DPGN) predict end-stage renal disease (ESRD) progression in FGN patients.

Area of Science:

  • Nephrology
  • Pathology

Background:

  • Fibrillary glomerulonephritis (FGN) is a rare kidney disease characterized by abnormal fibril deposition.
  • It is associated with poor renal outcomes and lacks specific treatments.
  • Identifying predictors of FGN progression is crucial for patient management.

Purpose of the Study:

  • To identify clinical and pathological predictors of outcomes in patients with FGN.
  • To analyze a multi-institutional cohort with centralized histopathology review.

Main Methods:

  • Retrospective analysis of 35 patients with biopsy-proven FGN (2015-2024).
  • Clinical data, comorbidities, laboratory parameters, and outcomes were collected.
  • Histological characteristics and DNAJB9 staining were analyzed.

Main Results:

  • The cohort had a mean age of 62.8 years, with a 2.8:1 female-to-male ratio.
  • Common comorbidities included diabetes mellitus (34.2%) and malignancy (20%).
  • 51% progressed to end-stage renal disease (ESRD) within a median of 39 months. Female sex, proteinuria, and diffuse proliferative glomerulonephritis (DPGN) were significant predictors of ESRD.

Conclusions:

  • FGN is linked to malignancy, autoimmune disease, and diabetes mellitus.
  • Prognosis for FGN remains poor.
  • Proteinuria, DPGN, and female sex significantly influence progression to ESRD.
Keywords:
DNAJB9fibrillary glomerulonephritishistopathology

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