{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T16:39:22Z","timestamp":1785515962990,"version":"3.56.0"},"reference-count":65,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2021,2,5]],"date-time":"2021-02-05T00:00:00Z","timestamp":1612483200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000781","name":"European Research Council","doi-asserted-by":"publisher","award":["ERC-2012-ADG_20120314 grant 322947"],"award-info":[{"award-number":["ERC-2012-ADG_20120314 grant 322947"]}],"id":[{"id":"10.13039\/501100000781","id-type":"DOI","asserted-by":"publisher"}]},{"name":"European Consortium for High-Throughput Research in Rare Kidney Diseases","award":["2012-305608"],"award-info":[{"award-number":["2012-305608"]}]},{"DOI":"10.13039\/501100001665","name":"Agence Nationale de la Recherche","doi-asserted-by":"publisher","award":["ANR-20-CE17-0017-01"],"award-info":[{"award-number":["ANR-20-CE17-0017-01"]}],"id":[{"id":"10.13039\/501100001665","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001665","name":"Agence Nationale de la Recherche","doi-asserted-by":"publisher","award":["ANR-17- CE17-0012-01"],"award-info":[{"award-number":["ANR-17- CE17-0012-01"]}],"id":[{"id":"10.13039\/501100001665","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Membranous nephropathy (MN) is a rare auto-immune disease where the glomerulus is targeted by circulating auto-antibodies mostly against podocyte antigens, which results in the formation of electron-dense immune complexes, activation of complement and massive proteinuria. MN is the most common cause of nephrotic syndrome in adults leading to severe thrombotic complications and kidney failure. This review is focused on the recent therapeutic and pathophysiological advances that occurred in the last two years. For a long time, we were lacking a head-to-head comparison between cyclophosphamide considered as the gold standard therapy and other medications, notably rituximab. Substantial progress has been achieved owing to three randomized controlled trials. MENTOR (Membranous Nephropathy Trial of Rituximab) and STARMEN (Sequential Therapy with Tacrolimus and Rituximab in Primary Membranous Nephropathy) conclusively established that calcineurin inhibitor-based regimens are slower to result in an immunologic response than rituximab or cyclophosphamide, achieve fewer complete clinical remissions, and are less likely to maintainremission. Rituximab Versus Steroids and Cyclophosphamide in the Treatment of Idiopathic Membranous Nephropathy (RI-CYCLO) suggested that competition between cyclophosphamide and rituximab remains open. Given the technological leap combining laser microdissection of glomeruli and mass spectrometry of solubilized digested proteins, four \u201cnew antigens\u201d were discovered including NELL-1 and Semaphorin 3B in so-called primary MN, and exostosins 1 and 2 and NCAM 1 in lupus MN. NELL-1 is associated with about 8% of primary MN and is characterized by segmental immune deposits and frequent association with cancer (30%). Semaphorin 3B-associated MN usually occurs in children, often below the age of two years, where it is the main antigen, representing about 16% of non-lupus MN in childhood. Exostosins 1\/2 and NCAM 1 are associated with 30% and 6% of lupus MN, respectively. Exostosins 1\/2 (EXT1\/2) staining is associated with a low rate of end-stage kidney disease (ESKD) even in mixed classes III\/IV+V. These findings already lead to revisiting the diagnostic and therapeutic algorithms toward more personalized medicine.<\/jats:p>","DOI":"10.3390\/jcm10040607","type":"journal-article","created":{"date-parts":[[2021,2,7]],"date-time":"2021-02-07T14:04:13Z","timestamp":1612706653000},"page":"607","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":75,"title":["Advances in Membranous Nephropathy"],"prefix":"10.3390","volume":"10","author":[{"given":"Pierre","family":"Ronco","sequence":"first","affiliation":[{"name":"Unit\u00e9 Mixte de Recherche S1155, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, Sorbonne Universit\u00e9, Universit\u00e9 Pierre et Marie Curie Paris 06, H\u00f4pital Tenon, 75020 Paris, France"},{"name":"Reference Center of Rare Disease-Idiopathic Nephrotic Syndrome, H\u00f4pital Tenon, 75020 Paris, France"},{"name":"Department of Nephrology, Centre Hospitalier du Mans, 72000 Le Mans, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuelle","family":"Plaisier","sequence":"additional","affiliation":[{"name":"Unit\u00e9 Mixte de Recherche S1155, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, Sorbonne Universit\u00e9, Universit\u00e9 Pierre et Marie Curie Paris 06, H\u00f4pital Tenon, 75020 Paris, France"},{"name":"Reference Center of Rare Disease-Idiopathic Nephrotic Syndrome, H\u00f4pital Tenon, 75020 Paris, France"},{"name":"Day Hospital of Nephrology, H\u00f4pital Tenon, Assistance Publique-H\u00f4pitaux de Paris, 75020 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanna","family":"Debiec","sequence":"additional","affiliation":[{"name":"Unit\u00e9 Mixte de Recherche S1155, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, Sorbonne Universit\u00e9, Universit\u00e9 Pierre et Marie Curie Paris 06, H\u00f4pital Tenon, 75020 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,2,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1983","DOI":"10.1016\/S0140-6736(15)60731-0","article-title":"Pathophysiological advances in membranous nephropathy: Time for a shift in patient\u2019s care","volume":"385","author":"Ronco","year":"2015","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1600","DOI":"10.1038\/ki.1995.453","article-title":"A 10-year follow-up of a randomized study with methylprednisolone and chlorambucil in membranous nephropathy","volume":"48","author":"Ponticelli","year":"1995","journal-title":"Kidney Int."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1899","DOI":"10.1681\/ASN.2007020166","article-title":"A randomized, controlled trial of steroids and cyclophosphamide in adults with nephrotic syndrome caused by idiopathic membranous nephropathy","volume":"18","author":"Jha","year":"2007","journal-title":"J. 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