TY - JOUR T1 - Fibrillary glomerulonephritis in a patient with systemic lupus erythematosus with no evidence of lupus nephritis JF - BMJ Case Reports JO - BMJ Case Reports DO - 10.1136/bcr-2022-253388 VL - 16 IS - 2 SP - e253388 AU - Matthew Carl Whelband AU - Tom Willingham AU - Sathiamalar Thirunavukkarasu AU - Jean Patrick Y1 - 2023/02/01 UR - http://casereports.bmj.com/content/16/2/e253388.abstract N2 - 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. ER -