{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T06:17:29Z","timestamp":1792736249722,"version":"4.5.0"},"reference-count":35,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","funder":[{"DOI":"10.13039\/100011102","name":"Seventh Framework Programme","doi-asserted-by":"publisher","award":["2012-305608"],"award-info":[{"award-number":["2012-305608"]}],"id":[{"id":"10.13039\/100011102","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010281","name":"Kidneeds","doi-asserted-by":"publisher","award":["2015"],"award-info":[{"award-number":["2015"]}],"id":[{"id":"10.13039\/100010281","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001665","name":"Agence Nationale de la Recherche","doi-asserted-by":"publisher","award":["ANR-16-CE18-0015-01"],"award-info":[{"award-number":["ANR-16-CE18-0015-01"]}],"id":[{"id":"10.13039\/501100001665","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002915","name":"Fondation pour la Recherche M\u00e9dicale","doi-asserted-by":"crossref","award":["FDM 20130727355"],"award-info":[{"award-number":["FDM 20130727355"]}],"id":[{"id":"10.13039\/501100002915","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JASN"],"published-print":{"date-parts":[[2020,4]]},"abstract":"\n Significance Statement<\/jats:title>\n Acute postinfectious GN, the leading cause of acute nephritis in children, associates with transient complement activation of undetermined mechanism. Its clinical features overlap considerably with those of C3 glomerulopathy, a severe chronic condition. In this retrospective study, the authors demonstrated that in more than 90% of children with acute postinfectious GN, complement overactivation results from activation of the alternative pathway of the complement system, driven by transient presence of autoantibodies targeting factor B, a component of the alternative C3 convertase. They also identified crucial antibody binding sites on factor B, including one correlated to disease severity at onset. The presence of anti-factor B antibodies was highly specific to acute postinfectious GN, suggesting that screening for these antibodies might help clinicians distinguish the disorder during its acute phase from C3 glomerulopathy.<\/jats:p>\n <\/jats:sec>\n \n Background<\/jats:title>\n The pathophysiology of the leading cause of pediatric acute nephritis, acute postinfectious GN, including mechanisms of the pathognomonic transient complement activation, remains uncertain. It shares clinicopathologic features with C3 glomerulopathy, a complement-mediated glomerulopathy that, unlike acute postinfectious GN, has a poor prognosis.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n This retrospective study investigated mechanisms of complement activation in 34 children with acute postinfectious GN and low C3 level at onset. We screened a panel of anticomplement protein autoantibodies, carried out related functional characterization, and compared results with those of 60 children from the National French Registry who had C3 glomerulopathy and persistent hypocomplementemia.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n All children with acute postinfectious GN had activation of the alternative pathway of the complement system. At onset, autoantibodies targeting factor B (a component of the alternative pathway C3 convertase) were found in a significantly higher proportion of children with the disorder versus children with hypocomplementemic C3 glomerulopathy (31 of 34 [91%] versus 4 of 28 [14%], respectively). In acute postinfectious GN, anti-factor B autoantibodies were transient and correlated with plasma C3 and soluble C5b-9 levels. We demonstrated that anti-factor B antibodies enhance alternative pathway convertase activity\n in vitro<\/jats:italic>\n , confirming their pathogenic effect. We also identified crucial antibody binding sites on factor B, including one correlated to disease severity.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n These findings elucidate the pathophysiologic mechanisms underlying acute postinfectious GN by identifying anti-factor B autoantibodies as contributing factors in alternative complement pathway activation. At onset of a nephritic syndrome with low C3 level, screening for anti-factor B antibodies might help guide indications for kidney biopsy to avoid misdiagnosed chronic glomerulopathy, such as C3 glomerulopathy, and to help determine therapy.<\/jats:p>\n <\/jats:sec>","DOI":"10.1681\/asn.2019080851","type":"journal-article","created":{"date-parts":[[2020,2,7]],"date-time":"2020-02-07T18:15:12Z","timestamp":1581099312000},"page":"829-840","source":"Crossref","is-referenced-by-count":90,"title":["Anti-Factor B Antibodies and Acute Postinfectious GN in Children"],"prefix":"10.1681","volume":"31","author":[{"given":"Sophie","family":"Chauvet","sequence":"first","affiliation":[{"name":"Inflammation, Complement and Cancer Team, Cordeliers Research Center, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale (INSERM) Unit\u00e9 Mixte de Recherche (UMR) S1138, Paris, France;"},{"name":"Nephrology and"},{"name":"Paris University, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romain","family":"Berthaud","sequence":"additional","affiliation":[{"name":"Paris University, Paris, France;"},{"name":"Department of Pediatric Nephrology, AP-HP, Necker Hospital \u2013 Sick Children, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Magali","family":"Devriese","sequence":"additional","affiliation":[{"name":"Immunology, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Georges Pompidou European Hospital, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Morgane","family":"Mignotet","sequence":"additional","affiliation":[{"name":"Immunology, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Georges Pompidou European Hospital, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paula","family":"Vieira Martins","sequence":"additional","affiliation":[{"name":"Immunology, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Georges Pompidou European Hospital, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tania","family":"Robe-Rybkine","sequence":"additional","affiliation":[{"name":"Inflammation, Complement and Cancer Team, Cordeliers Research Center, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale (INSERM) Unit\u00e9 Mixte de Recherche (UMR) S1138, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria A.","family":"Miteva","sequence":"additional","affiliation":[{"name":"Paris University, Paris, France;"},{"name":"INSERM U1268 Medicinal Chemistry and Translational Research, Cibles Th\u00e9rapeutiques et Conception du M\u00e9dicament UMR8038 Centre National de la Recherche Scientifique (CNRS), Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aram","family":"Gyulkhandanyan","sequence":"additional","affiliation":[{"name":"University of Paris Diderot, Sorbonne Paris Cit\u00e9, 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