{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2022,8,28]],"date-time":"2022-08-28T23:11:45Z","timestamp":1661728305168},"reference-count":19,"publisher":"Georg Thieme Verlag KG","issue":"17","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dtsch Med Wochenschr"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:title>Was ist neu?<\/jats:title><jats:p>\n          Pathogenese der MGN\u2002Die Identifikation der f\u00fcr die Entstehung der MGN verantwortlichen Autoantik\u00f6rper erm\u00f6glicht es, die Diagnose und Therapie an die molekulare Pathogenese der Erkrankung anzupassen. Die zugrunde liegenden Mechanismen der PLA2R1-Antik\u00f6rper-Entstehung sind noch unklar. Umweltfaktoren wie Luftverschmutzung sowie genetische Faktoren spielen m\u00f6glicherweise eine wichtige Rolle. Bei der THSD7A-assoziierten MGN k\u00f6nnen THSD7A-exprimierende Tumore zur Entstehung der THSD7A-Antik\u00f6rper beitragen.<\/jats:p><jats:p>\n          Klinische Anwendung\u2002Eine antigenspezifische Diagnose ist hilfreich, um die anschlie\u00dfende Differenzialdiagnostik der MGN, z.\u200aB. eine Tumorsuche, zu individualisieren. PLA2R1-Antik\u00f6rper sind sehr spezifisch f\u00fcr die Diagnose einer MGN und erm\u00f6glichen eine personalisierte Entscheidung f\u00fcr oder gegen eine Nierenbiopsie zur Diagnosesicherung, abh\u00e4ngig von der klinischen und laborchemischen Pr\u00e4sentation der Patienten. Die PLA2R1-Antik\u00f6rper erm\u00f6glichen eine st\u00e4rkere Anpassung der Therapie an die Pathogenese und der immunologischen Aktivit\u00e4t der MGN. Der PLA2R1-Antik\u00f6rperspiegel wird sowohl zur Risikoeinteilung als auch f\u00fcr Therapie-Entscheidungen genutzt.<\/jats:p><jats:p>\n          PLA2R1-Antik\u00f6rperbestimmung\u2002PLA2R1-Antik\u00f6rper k\u00f6nnen gegen verschiede Bindungsstellen am PLA2R1 gerichtet sein. Aktuell ist im klinischen Alltag die Bestimmung der Gesamt-PLA2R1-Antik\u00f6rperspiegel entscheidend f\u00fcr die Prognose und Therapie\u00fcberwachung.<\/jats:p><jats:p>\n          Neue potenzielle Antigene der MGN\u2002In den letzten Jahren wurde eine Vielzahl weiterer potenzieller Antigene bei Patienten mit MGN beschrieben. Ihre pathophysiologische Rolle ist noch nicht gekl\u00e4rt. Je nach beschriebenem Antigen wurden Assoziationen der MGN mit verschiedenen Erkrankungen berichtet.<\/jats:p><jats:p>\n          Pathogenese der MCD und FSGS\u2002bleibt ungekl\u00e4rt. Eine T-Zell-St\u00f6rung wurde seit Langem als m\u00f6gliche Ursache angenommen, w\u00e4hrend in den letzten Jahren auch f\u00fcr B-Zellen und Autoantik\u00f6rper zunehmend eine pathogenetische Rolle postuliert wird.<\/jats:p><jats:p>\n          Diagnose und Therapie\u2002F\u00fcr die Diagnose einer MCD und FSGS bei Erwachsenen ist die Nierenbiopsie obligat. In der Therapie der prim\u00e4ren MCD und FSGS bleibt auch in den neuen KDIGO-Therapie-Empfehlungen die Steroidtherapie die 1.\u00a0Wahl. In der Zweitlinientherapie werden vor allem Calcineurin-Inhibitoren eingesetzt. Als weitere Therapieoptionen gelten Cyclophosphamid, MMF und Rituximab.<\/jats:p>","DOI":"10.1055\/a-1585-3260","type":"journal-article","created":{"date-parts":[[2022,8,28]],"date-time":"2022-08-28T22:30:26Z","timestamp":1661725826000},"page":"1134-1140","source":"Crossref","is-referenced-by-count":0,"title":["Update nephrotisches Syndrom \u2013 neue pathophysiologische Konzepte 2022"],"prefix":"10.1055","volume":"147","author":[{"given":"Elion","family":"Hoxha","sequence":"additional","affiliation":[{"name":"III. Medizinische Klinik und Poliklinik, Universit\u00e4tsklinikum Hamburg-Eppendorf"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2022,8,28]]},"reference":[{"issue":"04","key":"ref1","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1038\/ki.2010.156","article-title":"Glomerular disease: why is there a dearth of high quality clinical trials?","volume":"78","author":"D E Leaf","year":"2010","journal-title":"Kidney Int"},{"issue":"01","key":"ref2","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1038\/s41572-021-00303-z","article-title":"Membranous nephropathy","volume":"7","author":"P Ronco","year":"2021","journal-title":"Nat Rev Dis Primers"},{"issue":"S 04","key":"ref3","first-page":"S1","article-title":"KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases","volume":"100","author":"Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group","year":"2021","journal-title":"Kidney Int"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1080\/1744666X.2019.1548934","article-title":"Characterization of autoantibodies in primary membranous nephropathy and their clinical significance","volume":"15","author":"R AK Stahl","year":"2019","journal-title":"Expert Rev Clin Immunol"},{"issue":"01","key":"ref5","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1681\/ASN.2019050538","article-title":"Detection of PLA2R Autoantibodies before the Diagnosis of Membranous Nephropathy","volume":"31","author":"P D Burbelo","year":"2020","journal-title":"J Am Soc Nephrol"},{"issue":"10","key":"ref6","doi-asserted-by":"crossref","first-page":"e110681","DOI":"10.1371\/journal.pone.0110681","article-title":"PLA2R antibody levels and clinical outcome in patients with membranous nephropathy and non-nephrotic range proteinuria under treatment with inhibitors of the renin-angiotensin system","volume":"9","author":"E Hoxha","year":"2014","journal-title":"PLoS One"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"1292","DOI":"10.1016\/j.kint.2019.07.014","article-title":"The anti-PLA2R antibody in membranous nephropathy: what we know and what remains a decade after its discovery","volume":"96","author":"A E van de Logt","year":"2019","journal-title":"Kidney Int"},{"issue":"01","key":"ref8","doi-asserted-by":"crossref","first-page":"1600","DOI":"10.1038\/s41467-020-15383-w","article-title":"The genetic architecture of membranous nephropathy and its potential to improve non-invasive diagnosis","volume":"11","author":"J Xie","year":"2020","journal-title":"Nat Commun"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1016\/j.kint.2020.08.007","article-title":"HLA-D and PLA2R1 risk alleles associate with recurrent primary membranous nephropathy in kidney transplant recipients","volume":"99","author":"L Berchtold","year":"2021","journal-title":"Kidney Int"},{"issue":"02","key":"ref10","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1681\/ASN.2016070776","article-title":"A Proposal for a Serology-Based Approach to Membranous Nephropathy","volume":"28","author":"A S De Vriese","year":"2017","journal-title":"J Am Soc Nephrol"},{"issue":"02","key":"ref11","doi-asserted-by":"crossref","first-page":"520","DOI":"10.1681\/ASN.2016010050","article-title":"An Indirect Immunofluorescence Method Facilitates Detection of Thrombospondin Type 1 Domain-Containing 7A-Specific Antibodies in Membranous Nephropathy","volume":"28","author":"E Hoxha","year":"2017","journal-title":"J Am Soc Nephrol"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"800242","DOI":"10.3389\/fimmu.2021.800242","article-title":"How Times Have Changed! 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