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Case Reports: Rare disease
Fibrillary glomerulonephritis in a patient with systemic lupus erythematosus with no evidence of lupus nephritis
  1. Matthew Carl Whelband1,
  2. Tom Willingham2,
  3. Sathiamalar Thirunavukkarasu2 and
  4. Jean Patrick3
  1. 1Acute Medicine, James Paget University Hospitals NHS Foundation Trust, Great Yarmouth, Norfolk, UK
  2. 2Department of Histopathology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, Cambridgeshire, UK
  3. 3Nephrology, James Paget University Hospitals NHS Foundation Trust, Great Yarmouth, Norfolk, UK
  1. Correspondence to Dr Matthew Carl Whelband; matthew.whelband{at}jpaget.nhs.uk

Abstract

Fibrillary glomerulonephritis (FGN) is a rare proliferative form of glomerular disease characterised by randomly oriented fibrillary deposits with a mean diameter of 20 nm. It has a rare association with systemic lupus erythematosus (SLE). We report the case of a female in her mid-50's with a 20 year history of SLE, who developed proteinuria due to FGN and had no histological evidence of lupus nephritis. She was maintained on azathioprine and prednisolone. A renal biopsy revealed randomly arranged fibrillary deposits that positively stained for DNAJB9, consistent with a diagnosis of FGN. Azathioprine was switched to mycophenolate mofetil, and the patient showed significant improvement in proteinuria. This case-based review describes the diagnosis, management and clinical outcome of FGN in association with SLE in the absence of lupus nephritis.

  • Biological agents
  • Pathology
  • Renal medicine
  • Systemic lupus erythematosus

https://doi.org/10.1136/bcr-2022-253388

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    Footnotes

    • Contributors MW was responsible for reviewing the source material and literature and writing the manuscript. TW was responsible for preparing renal biopsy slides and figures presented in the manuscript. ST performed the biopsy and reviewed the histopathology details of the manuscript.JP supervised and reviewed the manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.