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 |
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Erscheinungsjahr: |
2023 |
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Erschienen: |
2023 |
Enthalten in: |
Zur Gesamtaufnahme - volume:16 |
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Enthalten in: |
BMJ case reports - 16(2023), 2 vom: 21. Feb. |
Sprache: |
Englisch |
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Beteiligte Personen: |
Whelband, Matthew Carl [VerfasserIn] |
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Links: |
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Anmerkungen: |
Date Completed 24.02.2023 Date Revised 24.02.2023 published: Electronic Citation Status MEDLINE |
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doi: |
10.1136/bcr-2022-253388 |
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funding: |
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Förderinstitution / Projekttitel: |
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PPN (Katalog-ID): |
NLM353166421 |
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520 | |a 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 | ||
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