PT  - JOURNAL ARTICLE
AU  - Whelband, Matthew Carl
AU  - Willingham, Tom
AU  - Thirunavukkarasu, Sathiamalar
AU  - Patrick, Jean
TI  - Fibrillary glomerulonephritis in a patient with systemic lupus erythematosus with no evidence of lupus nephritis
AID  - 10.1136/bcr-2022-253388
DP  - 2023 Feb 01
TA  - BMJ Case Reports
PG  - e253388
VI  - 16
IP  - 2
4099  - http://casereports.bmj.com/content/16/2/e253388.short
4100  - http://casereports.bmj.com/content/16/2/e253388.full
SO  - BMJ Case Reports2023 Feb 01; 16
AB  - 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.