NELL-1 positive membranous nephropathy in association with antisynthetase syndrome

Journal article

Membranous nephropathy (MN) is an important cause of nephrotic syndrome and is associated with significant adverse health outcomes including progression to end-stage kidney disease, complications relating to volume overload and increased risk of venous thromboembolism. Primary MN is frequently linked to antibodies against the phospholipase A2 receptor, although a broader range of target autoantigens are emerging. We report a case of a man in his mid-60s who had been recently diagnosed with antisynthetase syndrome presenting with nephrotic syndrome. A kidney biopsy revealed findings in keeping with MN, including positive immunohistochemical staining for the neural epidermal growth factor-like 1 protein (NELL-1) autoantigen. This report highlights a possible novel association between antisynthetase syndrome and NELL-1 positive MN.

Authors: Zacharias, AP; Hoque, MA; Swift, O; Mathavakkannan, S

• Publication status: Published
• Peer review status: Peer reviewed
• Publisher: BMJ Publishing Group
• Journal: BMJ Case Reports
• Volume: 18
• Issue: 11
• Article number: e267264
• Publication date: 2025-11-12
• Acceptance date: 2025-10-31
• DOI: 10.1136/bcr-2025-267264
• EISSN: 1757-790X
• Pmid: 41224692
• Language: English
• Keywords: nephrotic syndrome; autoantibodies; biopsy; immunohistochemistry; kidney; glomerulonephritis; membranous; nerve tissue proteins; musculoskeletal syndromes; myositis; interstitial lung disease