Fibrillar glomerulonephritis in a patient with systemic lupus erythematosus with no evidence of lupus nephritis. Issue 2 (21st February 2023)

• Record Type: Journal Article
• Authors: Whelband, Matthew Carl; Willingham, Tom; Thirunavukkarasu, Sathiamalar; Patrick, Jean
• Is Part Of: BMJ case reports. Volume 16:Issue 2(2023)
• Publication Date: 2023-02-21
• Subjects: Biological agents -- Pathology -- Renal medicine -- Systemic lupus erythematosus; Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2022-253388
• Languages: English
• ISSNs: 1757-790X

Abstract

Abstract : 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.