RT Journal Article
SR Electronic
T1 Fibrillary glomerulonephritis in a patient with systemic lupus erythematosus with no evidence of lupus nephritis
JF BMJ Case Reports
JO BMJ Case Reports
FD BMJ Publishing Group Ltd
SP e253388
DO 10.1136/bcr-2022-253388
VO 16
IS 2
A1 Matthew Carl Whelband
A1 Tom Willingham
A1 Sathiamalar Thirunavukkarasu
A1 Jean Patrick
YR 2023
UL http://casereports.bmj.com/content/16/2/e253388.abstract
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.