Fibrillar glomerulonephritis in a patient with systemic lupus erythematosus with no evidence of lupus nephritis

© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ..

Fibrillar glomerulonephritis (FGN) is a rare proliferative form of glomerular disease characterised by randomly oriented fibrillar 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 fibrillar 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.

Medienart:

E-Artikel

Erscheinungsjahr:

2023

Erschienen:

2023

Enthalten in:

Zur Gesamtaufnahme - volume:16

Enthalten in:

BMJ case reports - 16(2023), 2 vom: 21. Feb.

Sprache:

Englisch

Beteiligte Personen:

Whelband, Matthew Carl [VerfasserIn]
Willingham, Tom [VerfasserIn]
Thirunavukkarasu, Sathiamalar [VerfasserIn]
Patrick, Jean [VerfasserIn]

Links:

Volltext

Themen:

Azathioprine
Biological agents
Case Reports
DNAJB9 protein, human
HSP40 Heat-Shock Proteins
Journal Article
MRK240IY2L
Membrane Proteins
Molecular Chaperones
Pathology
Renal medicine
Systemic lupus erythematosus

Anmerkungen:

Date Completed 24.02.2023

Date Revised 24.02.2023

published: Electronic

Citation Status MEDLINE

doi:

10.1136/bcr-2022-253388

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM353166421