{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,25]],"date-time":"2025-09-25T13:57:05Z","timestamp":1758808625573},"reference-count":23,"publisher":"Springer Science and Business Media LLC","issue":"S5","license":[{"start":{"date-parts":[[2023,8,1]],"date-time":"2023-08-01T00:00:00Z","timestamp":1690848000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2023,9,20]],"date-time":"2023-09-20T00:00:00Z","timestamp":1695168000000},"content-version":"vor","delay-in-days":50,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"University of Innsbruck and Medical University of Innsbruck"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Wien Klin Wochenschr"],"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>Die membranoproliferative Glomerulonephritis (MPGN) repr\u00e4sentiert eine heterogene Gruppe von Erkrankungen. Das gemeinsame Merkmal eines membranoproliferativen L\u00e4sionsmusters in der Histologie der Nierenbiopsie kann sowohl idiopathisch\/prim\u00e4r auftreten, als auch \u2013\u00a0wesentlich h\u00e4ufiger\u00a0\u2013 eine sekund\u00e4re Ursache haben. Die historische licht- und elektronenmikroskopische Einteilung in MPGN Typ\u00a0I bis\u00a0III wurde weitgehend verlassen und in den letzten Jahren durch eine Pathogenese-orientierte Einteilung ersetzt. Von einer Immunkomplex-GN (IK-GN) spricht man beim Vorliegen einer MPGN mit C1q, C3 und\/oder C4 Ablagerungen, w\u00e4hrend eine MPGN mit dominanten C3-Ablagerungen als C3-Glomerulopathie (C3G) bezeichnet wird. Diese wird wiederum in eine C3-Glomerulonephritis (C3GN) und eine dense-deposit disease (DDD) eingeteilt. Diese Diagnosen k\u00f6nnen nur durch eine Nierenbiopsie gestellt werden. M\u00f6gliche Ursachen f\u00fcr eine MPGN sind chronische Infektionen (v.\u202fa. Hepatitis\u00a0B und\u00a0C, bakterielle Infektionen, Infektionen mit Protozoen). Autoimmunerkrankungen (v.\u202fa. Lupus, rheumatoide Arthritis) oder Malignome (v.\u202fa. h\u00e4matologische maligne Erkrankungen). Insbesondere im Falle einer C3G wird auch eine umfassende Abkl\u00e4rung des Komplementystems empfohlen. Therapeutische Entscheidungen sind aufgrund der niedrigen Inzidenz und des heterogenen klinischen Erscheinungsbildes einer MPGN individuell zu treffen, eine optimale generelle Therapie ist unbekannt. Im Falle einer identifizierten Ursache einer MPGN wird prinzipiell empfohlen diese zu behandeln, ebenso sollte die supportive Therapie, wie auch bei anderen Glomerulonephritiden optimiert werden. Bei anhaltender signifikanter Proteinurie und einer eGFR &gt;\u202f30\u202fml\/min\/1,73\u202fm<jats:sup>2<\/jats:sup> wird eine Therapie mit systemischen Steroiden und Mycophenolat Mofetil empfohlen. Weitere Therapieoptionen sind Rituximab und Eculizumab. Eine rapid-progressive MPGN sollte wie eine ANCA-assoziierte Vaskulitis therapiert werden. Die Rekurrenzraten nach einer Nierentransplantation sind sehr hoch und therapeutisch herausfordernd.<\/jats:p>","DOI":"10.1007\/s00508-023-02264-7","type":"journal-article","created":{"date-parts":[[2023,9,20]],"date-time":"2023-09-20T13:01:56Z","timestamp":1695214916000},"page":"688-695","update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Diagnose und Therapie glomerul\u00e4rer Erkrankungen mit einem membranoproliferativen L\u00e4sionsmuster (MPGN)\u00a0\u2013 2023","Diagnosis and treatment of glomerular diseases with a membranoproliferative glomerulonephritis (MPGN) pattern of injury"],"prefix":"10.1007","volume":"135","author":[{"given":"Michael","family":"Rudnicki","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Windpessl","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kathrin","family":"Eller","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Balazs","family":"Odler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philipp","family":"Gauckler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Irmgard","family":"Neumann","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emanuel","family":"Zitt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Heinz","family":"Regele","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"Kronbichler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karl","family":"Lhotta","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marcus D.","family":"S\u00e4emann","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2023,9,20]]},"reference":[{"issue":"5","key":"2264_CR1","doi-asserted-by":"publisher","first-page":"1135","DOI":"10.1016\/j.kint.2020.05.053","volume":"98","author":"F Fakhouri","year":"2020","unstructured":"Fakhouri\u00a0F, Le Quintrec\u00a0M, Fremeaux-Bacchi\u00a0V. Practical management of C3 glomerulopathy and Ig-mediated MPGN: facts and uncertainties. Kidney Int. 2020;98(5):1135\u201348.","journal-title":"Kidney Int"},{"key":"2264_CR2","doi-asserted-by":"crossref","unstructured":"KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021;100(4S):S1\u2013S276.","DOI":"10.1016\/j.kint.2021.05.021"},{"issue":"2","key":"2264_CR3","doi-asserted-by":"publisher","first-page":"224","DOI":"10.1053\/j.ajkd.2018.12.046","volume":"74","author":"P Ruggenenti","year":"2019","unstructured":"Ruggenenti\u00a0P, Daina\u00a0E, Gennarini\u00a0A, Carrara\u00a0C, Gamba\u00a0S, Noris\u00a0M, et al. C5 Convertase blockade in membranoproliferative glomerulonephritis: a\u00a0single-arm clinical trial. Am J Kidney Dis. 2019;74(2):224\u201338.","journal-title":"Am J Kidney Dis"},{"issue":"1","key":"2264_CR4","doi-asserted-by":"publisher","first-page":"84","DOI":"10.1053\/j.ajkd.2017.11.019","volume":"72","author":"M Le Quintrec","year":"2018","unstructured":"Le Quintrec\u00a0M, Lapeyraque\u00a0AL, Lionet\u00a0A, Sellier-Leclerc\u00a0AL, Delmas\u00a0Y, Baudouin\u00a0V, et al. Patterns of clinical response to Eculizumab in patients with C3 glomerulopathy. Am J Kidney Dis. 2018;72(1):84\u201392.","journal-title":"Am J Kidney Dis"},{"issue":"3","key":"2264_CR5","doi-asserted-by":"publisher","first-page":"406","DOI":"10.2215\/CJN.09080817","volume":"13","author":"RS Avasare","year":"2018","unstructured":"Avasare\u00a0RS, Canetta\u00a0PA, Bomback\u00a0AS, Marasa\u00a0M, Caliskan\u00a0Y, Ozluk\u00a0Y, et\u00a0al. Mycophenolate mofetil in combination with steroids for treatment of C3 glomerulopathy: a\u00a0case series. Clin J Am Soc Nephrol. 2018;13(3):406\u201313.","journal-title":"Clin J Am Soc Nephrol"},{"issue":"5","key":"2264_CR6","doi-asserted-by":"publisher","first-page":"1153","DOI":"10.1038\/ki.2015.227","volume":"88","author":"C Rabasco","year":"2015","unstructured":"Rabasco\u00a0C, Cavero\u00a0T, Roman\u00a0E, Rojas-Rivera\u00a0J, Olea\u00a0T, Espinosa\u00a0M, et al. Effectiveness of mycophenolate mofetil in C3 glomerulonephritis. Kidney Int. 2015;88(5):1153\u201360.","journal-title":"Kidney Int"},{"issue":"1","key":"2264_CR7","doi-asserted-by":"publisher","first-page":"22","DOI":"10.2215\/CJN.03480708","volume":"4","author":"SH Nasr","year":"2009","unstructured":"Nasr\u00a0SH, Valeri\u00a0AM, Appel\u00a0GB, Sherwinter\u00a0J, Stokes\u00a0MB, Said\u00a0SM, et\u00a0al. Dense deposit disease: clinicopathologic study of 32 pediatric and adult patients. Clin J Am Soc Nephrol. 2009;4(1):22\u201332.","journal-title":"Clin J Am Soc Nephrol"},{"issue":"9","key":"2264_CR8","doi-asserted-by":"publisher","first-page":"2447","DOI":"10.1681\/ASN.2007030356","volume":"18","author":"RJ Smith","year":"2007","unstructured":"Smith\u00a0RJ, Alexander\u00a0J, Barlow\u00a0PN, Botto\u00a0M, Cassavant\u00a0TL, Cook\u00a0HT, et\u00a0al. New approaches to the treatment of dense deposit disease. J\u00a0Am Soc Nephrol. 2007;18(9):2447\u201356.","journal-title":"J Am Soc Nephrol"},{"issue":"12","key":"2264_CR9","doi-asserted-by":"publisher","first-page":"1119","DOI":"10.1056\/NEJMra1108178","volume":"366","author":"S Sethi","year":"2012","unstructured":"Sethi\u00a0S, Fervenza\u00a0FC. Membranoproliferative glomerulonephritis\u2014a new look at an old entity. N\u00a0Engl J Med. 2012;366(12):1119\u201331.","journal-title":"N Engl J Med"},{"key":"2264_CR10","volume-title":"Heptinstall\u2019s pathology of the kidney","author":"J Jennette","year":"2007","unstructured":"Jennette\u00a0J, Olson\u00a0J, Schwartz\u00a0M, Silva\u00a0F. Heptinstall\u2019s pathology of the kidney. 6.\u00a0Aufl. Lippincott Williams & Wilkins; 2007.","edition":"6"},{"issue":"11","key":"2264_CR11","doi-asserted-by":"publisher","first-page":"634","DOI":"10.1038\/nrneph.2012.213","volume":"8","author":"A Bomback","year":"2012","unstructured":"Bomback\u00a0A, Appel\u00a0G. Pathogenesis of the C3 glomerulopathies and reclassification of MPGN. Nat Rev Nephrol. 2012;8(11):634\u201342.","journal-title":"Nat Rev Nephrol"},{"issue":"1","key":"2264_CR12","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1038\/s41581-018-0077-4","volume":"15","author":"N Leung","year":"2019","unstructured":"Leung\u00a0N, Bridoux\u00a0F, Batuman\u00a0V, Chaidos\u00a0A, Cockwell\u00a0P, D\u2019Agati\u00a0VD, et\u00a0al. The evaluation of monoclonal gammopathy of renal significance: a\u00a0consensus report of the International Kidney and Monoclonal Gammopathy Research Group. Nat Rev Nephrol. 2019;15(1):45\u201359.","journal-title":"Nat Rev Nephrol"},{"issue":"6","key":"2264_CR13","doi-asserted-by":"publisher","first-page":"1079","DOI":"10.1038\/ki.2013.377","volume":"84","author":"MC Pickering","year":"2013","unstructured":"Pickering\u00a0MC, D\u2019Agati\u00a0VD, Nester\u00a0CM, Smith\u00a0RJ, Haas\u00a0M, Appel\u00a0GB, et\u00a0al. C3 glomerulopathy: consensus report. Kidney Int. 2013;84(6):1079\u201389.","journal-title":"Kidney Int"},{"issue":"3","key":"2264_CR14","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1136\/jmg.2006.045328","volume":"44","author":"A Servais","year":"2007","unstructured":"Servais\u00a0A, Fremeaux-Bacchi\u00a0V, Lequintrec\u00a0M, Salomon\u00a0R, Blouin\u00a0J, Knebelmann\u00a0B, et al. Primary glomerulonephritis with isolated C3 deposits: a\u00a0new entity which shares common genetic risk factors with haemolytic uraemic syndrome. J\u00a0Med Genet. 2007;44(3):193\u20139.","journal-title":"J Med Genet"},{"issue":"Suppl 2","key":"2264_CR15","first-page":"139","volume":"2","author":"Kidney Disease: Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group","year":"2012","unstructured":"Kidney Disease: Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group. KDIGO clinical practice guideline for glomerulonephritis. Kidney Int Suppl. 2012;2(Suppl 2):139\u2013274.","journal-title":"Kidney Int Suppl"},{"issue":"2","key":"2264_CR16","doi-asserted-by":"publisher","first-page":"140","DOI":"10.1007\/BF00860727","volume":"9","author":"M Somers","year":"1995","unstructured":"Somers M, Kertesz S, Rosen S, Herrin J, Colvin R, Palacios de Carreta N, et al. Non-nephrotic children with membranoproliferative glomerulonephritis: are steroids indicated? Pediatr Nephrol. 1995;9(2):140\u20134.","journal-title":"Pediatr Nephrol"},{"issue":"3","key":"2264_CR17","doi-asserted-by":"publisher","first-page":"539","DOI":"10.1016\/j.kint.2016.10.005","volume":"91","author":"TH Goodship","year":"2017","unstructured":"Goodship\u00a0TH, Cook\u00a0HT, Fakhouri\u00a0F, Fervenza\u00a0FC, Fremeaux-Bacchi\u00a0V, Kavanagh\u00a0D, et al. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a\u00a0\u201cKidney Disease: Improving Global Outcomes\u201d (KDIGO) Controversies Conference. Kidney Int. 2017;91(3):539\u201351.","journal-title":"Kidney Int"},{"issue":"1","key":"2264_CR18","doi-asserted-by":"publisher","first-page":"46","DOI":"10.2215\/CJN.04700513","volume":"9","author":"NR Medjeral-Thomas","year":"2014","unstructured":"Medjeral-Thomas\u00a0NR, O\u2019Shaughnessy\u00a0MM, O\u2019Regan\u00a0JA, Traynor\u00a0C, Flanagan\u00a0M, Wong\u00a0L, et al. C3 glomerulopathy: clinicopathologic features and predictors of outcome. Clin J Am Soc Nephrol. 2014;9(1):46\u201353.","journal-title":"Clin J Am Soc Nephrol"},{"key":"2264_CR19","doi-asserted-by":"publisher","first-page":"2180508","DOI":"10.1155\/2017\/2180508","volume":"2017","author":"M Rudnicki","year":"2017","unstructured":"Rudnicki\u00a0M. Rituximab for treatment of membranoproliferative glomerulonephritis and C3 glomerulopathies. Biomed Res Int. 2017;2017:2180508.","journal-title":"Biomed Res Int"},{"issue":"3","key":"2264_CR20","doi-asserted-by":"publisher","first-page":"835","DOI":"10.1002\/art.34322","volume":"64","author":"MC Sneller","year":"2012","unstructured":"Sneller\u00a0MC, Hu\u00a0Z, Langford\u00a0CA. A\u00a0randomized controlled trial of rituximab following failure of antiviral therapy for hepatitis C virus-associated cryoglobulinemic vasculitis. Arthritis Rheum. 2012;64(3):835\u201342.","journal-title":"Arthritis Rheum"},{"issue":"3","key":"2264_CR21","doi-asserted-by":"publisher","first-page":"843","DOI":"10.1002\/art.34331","volume":"64","author":"S De Vita","year":"2012","unstructured":"De Vita\u00a0S, Quartuccio\u00a0L, Isola\u00a0M, Mazzaro\u00a0C, Scaini\u00a0P, Lenzi\u00a0M, et\u00a0al. A\u00a0randomized controlled trial of rituximab for the treatment of severe cryoglobulinemic vasculitis. Arthritis Rheum. 2012;64(3):843\u201353.","journal-title":"Arthritis Rheum"},{"issue":"8","key":"2264_CR22","doi-asserted-by":"publisher","first-page":"1234","DOI":"10.1093\/rheumatology\/kem132","volume":"46","author":"D Saadoun","year":"2007","unstructured":"Saadoun\u00a0D, Landau\u00a0DA, Calabrese\u00a0LH, Cacoub\u00a0PP. Hepatitis\u00a0C-associated mixed cryoglobulinaemia: a\u00a0crossroad between autoimmunity and lymphoproliferation. Rheumatology. 2007;46(8):1234\u201342.","journal-title":"Rheumatology"},{"key":"2264_CR23","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1186\/s12882-015-0219-x","volume":"17","author":"S Alasfar","year":"2016","unstructured":"Alasfar\u00a0S, Carter-Monroe\u00a0N, Rosenberg\u00a0AZ, Montgomery\u00a0RA, Alachkar\u00a0N. Membranoproliferative glomerulonephritis recurrence after kidney transplantation: using the new classification. BMC Nephrol. 2016;17:7.","journal-title":"BMC Nephrol"}],"container-title":["Wiener klinische Wochenschrift"],"original-title":[],"language":"de","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00508-023-02264-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00508-023-02264-7\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00508-023-02264-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,20]],"date-time":"2023-09-20T13:18:11Z","timestamp":1695215891000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00508-023-02264-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,8]]},"references-count":23,"journal-issue":{"issue":"S5","published-print":{"date-parts":[[2023,8]]}},"alternative-id":["2264"],"URL":"https:\/\/doi.org\/10.1007\/s00508-023-02264-7","relation":{},"ISSN":["0043-5325","1613-7671"],"issn-type":[{"value":"0043-5325","type":"print"},{"value":"1613-7671","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,8]]},"assertion":[{"value":"24 July 2023","order":1,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 September 2023","order":2,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"M.\u00a0Rudnicki, M.\u00a0Windpessl, K.\u00a0Eller, B.\u00a0Odler, P.\u00a0Gauckler, I.\u00a0Neumann, E.\u00a0Zitt, H.\u00a0Regele, A.\u00a0Kronbichler, K.\u00a0Lhotta und M.D.\u00a0S\u00e4emann geben an, dass kein Interessenkonflikt besteht.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Interessenkonflikt"}}]}}