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After binding of the antibody to nephrin, a key component of the slit diaphragm, different intracellular signaling mechanisms lead to foot process effacement. These new insights into the pathophysiology of nephrotic syndrome may have the potential to influence clinical treatment strategies. First, the measurement provides a pathomechanism-specific serological biomarker of a previously clinically diagnosed renal disease. Second, it may have additional value for diagnosis and prognostication, as anti-nephrin antibodies are more common in steroid-sensitive forms and less frequently associated with multidrug-resistant forms. Furthermore, anti-nephrin autoantibodies have the potential to be used as biomarkers for disease activity and to estimate the risk of relapse before kidney transplantation, after conduction of appropriate clinical trials. Additionally, the detection of autoantibodies strengthens the role of B-cell targeting therapies and provides a potential rationale for further multicenter, prospective, randomized clinical trials using B-cell depletion to potentially reduce corticosteroid use and relapse rates in children. In the future, more targeted therapies such as the interference with downstream effects of antibody binding or the depletion of autoantigen-specific antibody producing cells might become possible. Finally, the detection of anti-nephrin autoantibodies provides an explanation for patients and their families with a previously elusive \u201cidiopathic\u201d disease in antibody positive patients. In summary, the identification of anti-nephrin antibodies in nephrotic syndrome now opens up new questions and paves the way for further experimental and clinical studies to improve diagnostic and therapeutic approaches.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00467-026-07292-9","type":"journal-article","created":{"date-parts":[[2026,4,23]],"date-time":"2026-04-23T18:43:02Z","timestamp":1776969782000},"page":"4281-4290","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Anti-nephrin antibodies in pediatric nephrotic syndrome: a new paradigm"],"prefix":"10.1007","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7984-2056","authenticated-orcid":false,"given":"Anne K.","family":"M\u00fchlig","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felicitas E.","family":"Hengel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun","family":"Oh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicola M.","family":"Tomas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tobias B.","family":"Huber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,4,23]]},"reference":[{"key":"7292_CR1","doi-asserted-by":"crossref","DOI":"10.1590\/1414-431x2022e11861","volume":"55","author":"LR Soares","year":"2022","unstructured":"Soares LR, Pantoja Junior JMS, Jorge LB, Yu L, Cavalcante LB, Malheiros D, Woronik V, Dias CB (2022) Nephrotic syndrome in the elderly: epidemiological aspects, clinical data, and renal biopsy findings. 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FEH reports honoraria from Novartis. TBH reports consultancy agreements with\u00a0Alexion, AstraZeneca, Bayer, Beren Therapeutics, Boehringer-Ingelheim, DaVita, Euroimmun, Fresenius Medical Care, Nipoka, Novartis, Otsuka, Pfizer, ProKidney, Protalix, Renovate, Sanofi, Travere, Vera Therapeutics, Vifor, and Vivoryon therapeutics; and\u00a0research funding from\u00a0Amicus Therapeutics, Fresenius Medical Care, and Euroimmun. TBH is Co-founder of Global Immune GmbH and holds the patents EP23154267.1, EP23154266.3, EP2015\/066881 and 3\/2024 PAT 1878 LU. NMT reports consulting fees from Merida Bioscience and honoraria from CSL Vifor, Novartis, and AstraZeneca. JO reports consulting fees from Boehringer Ingelheim, Novartis, Alexion, Pfizer, SOBI, and Fresenius. This work was supported by the Deutsche Forschungsgemeinschaft as part of the collaborative research center 1192 (to FH, AKM, TBH, and NMT), the CRC 1648 (TBH), the TRR422 (TBH, AKM, NMT), and CRC1453 (TBH), by the BMBF (STOP-FSGS-01GM2202A to TBH), by the Else Kr\u00f6ner Fresenius Foundation (Clinician Scientist Program iPRIME and Memorial Fellowship to FH and a Clinician Scientist Professorship to NMT), by the Else Kr\u00f6ner Fresenius Foundation and the Eva Luise und Horst K\u00f6hler Foundation (Clinician-Scientist-Program RECORD to FEH), and by the European Research Council (CureFSGS to TBH and AUTO-TARGET to NMT).","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}}]}}