{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T16:33:13Z","timestamp":1787761993886,"version":"build-2784847793"},"reference-count":36,"publisher":"BMJ","issue":"11","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2025,10,31]]},"published-print":{"date-parts":[[2025,11]]},"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.1136\/bcr-2025-267264","type":"journal-article","created":{"date-parts":[[2025,11,13]],"date-time":"2025-11-13T02:35:24Z","timestamp":1763001324000},"page":"e267264","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["NELL-1 positive membranous nephropathy in association with antisynthetase syndrome"],"prefix":"10.1136","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0560-5186","authenticated-orcid":false,"given":"Antony P","family":"Zacharias","sequence":"first","affiliation":[{"name":"Renal Medicine Department, East and North Hertfordshire Teaching NHS Trust, Stevenage, England, UK"},{"name":"Clinical and Professional Practice Unit, UCL Medical School, London, England, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-2886-3675","authenticated-orcid":false,"given":"Md Ariful","family":"Hoque","sequence":"additional","affiliation":[{"name":"Renal Medicine Department, East and North Hertfordshire Teaching NHS Trust, Stevenage, England, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oscar","family":"Swift","sequence":"additional","affiliation":[{"name":"Nephrology Department, Cambridge University Hospitals NHS Foundation Trust, Cambridge, England, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suresh","family":"Mathavakkannan","sequence":"additional","affiliation":[{"name":"Renal Medicine Department, East and North Hertfordshire Teaching NHS Trust, Stevenage, England, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2025,11,12]]},"reference":[{"key":"2025111218351596000_18.11.e267264.1","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0281795","article-title":"Membranous nephropathy in the UK Biobank","volume":"18","author":"Hamilton","year":"2023","journal-title":"PLoS One"},{"key":"2025111218351596000_18.11.e267264.2","doi-asserted-by":"crossref","first-page":"105","DOI":"10.25259\/ijn_317_23","article-title":"An Updated Review of Membranous Nephropathy","volume":"34","author":"Efe","year":"2024","journal-title":"Indian J Nephrol"},{"key":"2025111218351596000_18.11.e267264.3","unstructured":"Alok A , Yadav A . Membranous nephropathy. In: StatPearls. Treasure Island (FL): StatPearls Publishing, 2024. Available: http:\/\/www.ncbi.nlm.nih.gov\/books\/NBK559169\/"},{"key":"2025111218351596000_18.11.e267264.4","doi-asserted-by":"crossref","DOI":"10.1093\/ckj\/sfad228","article-title":"Novel biomarkers and pathophysiology of membranous nephropathy: PLA2R and beyond","volume":"17","author":"Murtas","year":"2024","journal-title":"Clin Kidney J"},{"key":"2025111218351596000_18.11.e267264.5","doi-asserted-by":"crossref","DOI":"10.3390\/biomedicines12102259","article-title":"An Updated Comprehensive Review on Diseases Associated with Nephrotic Syndromes","volume":"12","author":"Wendt","year":"2024","journal-title":"Biomedicines"},{"key":"2025111218351596000_18.11.e267264.6","doi-asserted-by":"publisher","DOI":"10.1016\/j.semnephrol.2013.08.004"},{"key":"2025111218351596000_18.11.e267264.7","doi-asserted-by":"publisher","DOI":"10.1016\/j.kint.2019.07.014"},{"key":"2025111218351596000_18.11.e267264.8","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1409354"},{"key":"2025111218351596000_18.11.e267264.9","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2020081165"},{"key":"2025111218351596000_18.11.e267264.10","doi-asserted-by":"crossref","DOI":"10.3389\/fmed.2020.611317","article-title":"Secondary Membranous Nephropathy. A Narrative Review","volume":"7","author":"Moroni","year":"2020","journal-title":"Front Med (Lausanne)"},{"key":"2025111218351596000_18.11.e267264.11","doi-asserted-by":"publisher","DOI":"10.1159\/000364782"},{"key":"2025111218351596000_18.11.e267264.12","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1093\/ckj\/sfac237","article-title":"The Many Faces of NELL1 MN","volume":"16","author":"Sethi","year":"2023","journal-title":"Clin Kidney J"},{"key":"2025111218351596000_18.11.e267264.13","doi-asserted-by":"crossref","DOI":"10.1136\/bmjresp-2023-002076","article-title":"Association of anti-Ro52 autoantibody with interstitial lung disease in autoimmune diseases: a systematic review and meta-analysis","volume":"10","author":"Nayebirad","year":"2023","journal-title":"BMJ Open Respir Res"},{"key":"2025111218351596000_18.11.e267264.14","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.221067"},{"key":"2025111218351596000_18.11.e267264.15","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2018080852"},{"key":"2025111218351596000_18.11.e267264.16","doi-asserted-by":"publisher","DOI":"10.1016\/j.kint.2019.09.014"},{"key":"2025111218351596000_18.11.e267264.17","doi-asserted-by":"publisher","DOI":"10.1016\/j.kint.2020.07.039"},{"key":"2025111218351596000_18.11.e267264.18","doi-asserted-by":"crossref","first-page":"2967","DOI":"10.1038\/s41598-022-07037-2","article-title":"Prevalence of neural epidermal growth factor-like 1- and exostosin 1\/exostosin 2-associated membranous nephropathy: a single-center retrospective study in Japan","volume":"12","author":"Iwakura","year":"2022","journal-title":"Sci Rep"},{"key":"2025111218351596000_18.11.e267264.19","unstructured":"Genetic and Rare Diseases Information Centre . Antisynthetase syndrome. Genetic and Rare Diseases Information Centre; 2024. Available: https:\/\/rarediseases.info.nih.gov\/diseases\/735\/antisynthetase-syndrome"},{"key":"2025111218351596000_18.11.e267264.20","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1016\/j.semarthrit.2020.09.020","article-title":"Antisynthetase syndrome - much more than just a myopathy","volume":"51","author":"Opinc","year":"2021","journal-title":"Semin Arthritis Rheum"},{"key":"2025111218351596000_18.11.e267264.21","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2017-211868"},{"key":"2025111218351596000_18.11.e267264.22","doi-asserted-by":"crossref","unstructured":"Son SH , Park MC , Kim S . Extracellular activities of aminoacyl-trna synthetases: new mediators for cell\u2013cell communication. In: Kim S , ed. Aminoacyl-tRNA synthetases in biology and medicine. Dordrecht: Springer Netherlands, 2014: 145\u201366. Available: https:\/\/doi.org\/10.1007\/128_2013_476","DOI":"10.1007\/128_2013_476"},{"key":"2025111218351596000_18.11.e267264.23","doi-asserted-by":"publisher","DOI":"10.1007\/s11926-013-0335-1"},{"key":"2025111218351596000_18.11.e267264.24","doi-asserted-by":"crossref","DOI":"10.3390\/jcm8112013","article-title":"Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course","volume":"8","author":"Cavagna","year":"2019","journal-title":"J Clin Med"},{"key":"2025111218351596000_18.11.e267264.25","doi-asserted-by":"publisher","DOI":"10.1002\/art.21883"},{"key":"2025111218351596000_18.11.e267264.26","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejim.2013.01.002"},{"key":"2025111218351596000_18.11.e267264.27","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1097\/MD.0000000000000015","article-title":"The spectrum of renal involvement in patients with inflammatory myopathies","volume":"93","author":"Couvrat-Desvergnes","year":"2014","journal-title":"Medicine (Baltimore)"},{"key":"2025111218351596000_18.11.e267264.28","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1093\/rheumatology\/kex500","article-title":"Scleroderma-like renal crisis in a patient with anti-threonyl-tRNA synthetase-associated antisynthetase syndrome","volume":"57","author":"Hermans","year":"2018","journal-title":"Rheumatology"},{"key":"2025111218351596000_18.11.e267264.29","doi-asserted-by":"crossref","first-page":"793","DOI":"10.1016\/j.kint.2019.05.024","article-title":"The immunohistological profile of membranous nephropathy associated with chronic Schistosoma mansoni infection reveals a glomerulopathy with primary features","volume":"96","author":"Ara\u00fajo","year":"2019","journal-title":"Kidney Int"},{"key":"2025111218351596000_18.11.e267264.30","doi-asserted-by":"publisher","DOI":"10.1038\/ki.2012.209"},{"key":"2025111218351596000_18.11.e267264.31","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1007\/s40620-017-0451-5","article-title":"Diagnostic specificity of autoantibodies to M-type phospholipase A2 receptor (PLA2R) in differentiating idiopathic membranous nephropathy (IMN) from secondary forms and other glomerular diseases","volume":"31","author":"Radice","year":"2018","journal-title":"J Nephrol"},{"key":"2025111218351596000_18.11.e267264.32","doi-asserted-by":"publisher","DOI":"10.1159\/000431331"},{"key":"2025111218351596000_18.11.e267264.33","doi-asserted-by":"crossref","first-page":"1424","DOI":"10.1016\/j.kint.2022.09.001","article-title":"Traditional indigenous medicines are an etiologic consideration for NELL1-positive membranous nephropathy","volume":"102","author":"Kurien","year":"2022","journal-title":"Kidney Int"},{"key":"2025111218351596000_18.11.e267264.34","doi-asserted-by":"crossref","first-page":"1208","DOI":"10.1016\/j.kint.2021.10.010","article-title":"Lipoic acid supplementation associated with neural epidermal growth factor-like 1 (NELL1)-associated membranous nephropathy","volume":"100","author":"Spain","year":"2021","journal-title":"Kidney Int"},{"key":"2025111218351596000_18.11.e267264.35","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1681\/ASN.0000000000000061","article-title":"Membranous Nephropathy in Syphilis is Associated with Neuron-Derived Neurotrophic Factor","volume":"34","author":"Sethi","year":"2023","journal-title":"J Am Soc Nephrol"},{"key":"2025111218351596000_18.11.e267264.36","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1016\/j.kint.2023.04.006","article-title":"Proprotein convertase subtilisin\/kexin type 6 (PCSK6) is a likely antigenic target in membranous nephropathy and nonsteroidal anti-inflammatory drug use","volume":"104","author":"Sethi","year":"2023","journal-title":"Kidney Int"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2025-267264","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,13]],"date-time":"2025-11-13T02:35:27Z","timestamp":1763001327000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2025-267264"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11]]},"references-count":36,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2025,11,12]]},"published-print":{"date-parts":[[2025,11]]}},"alternative-id":["10.1136\/bcr-2025-267264"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2025-267264","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,11]]}}}