{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T21:53:30Z","timestamp":1780437210515,"version":"3.54.1"},"reference-count":90,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2024,6,13]],"date-time":"2024-06-13T00:00:00Z","timestamp":1718236800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>C3 glomerulopathy is a rare disease, characterized by an abnormal activation of the complement\u2019s alternative pathway that leads to the accumulation of the C3 component in the kidney. The disease recurs in more than half of kidney transplant recipients, with a significant impact on graft survival. Recurrence of the primary disease represents the second cause of graft loss after organ rejection. In C3 glomerulopathy, there are several risk factors which can promote a recurrence during transplantation, such as delayed graft function, infection and monoclonal gammopathy. All these events can trigger the alternative complement pathway. In this review, we summarize the impact of C3 glomerulopathy on kidney grafts and present the latest treatment options. The most widely used treatments for the disease include corticosteroids and mycophenolate mofetil, which are already used chronically by kidney transplant recipients; thus, additional treatments for C3 glomerulopathy are required. Currently, several studies using anti-complement drugs (i.e., eculizumab, Ravalizumab, avacopan) for C3 glomerulopathy in kidney transplant patients are ongoing with encouraging results.<\/jats:p>","DOI":"10.3390\/ijms25126508","type":"journal-article","created":{"date-parts":[[2024,6,14]],"date-time":"2024-06-14T03:52:53Z","timestamp":1718337173000},"page":"6508","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Updates on C3 Glomerulopathy in Kidney Transplantation: Pathogenesis and Treatment Options"],"prefix":"10.3390","volume":"25","author":[{"given":"Giulia","family":"Bartoli","sequence":"first","affiliation":[{"name":"Department of Translational Medicine and Surgery, Universit\u00e0 Cattolica dl Sacro Cuore, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Dello Strologo","sequence":"additional","affiliation":[{"name":"Department of Translational Medicine and Surgery, Universit\u00e0 Cattolica dl Sacro Cuore, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Grandaliano","sequence":"additional","affiliation":[{"name":"Department of Translational Medicine and Surgery, Universit\u00e0 Cattolica dl Sacro Cuore, 00168 Rome, Italy"},{"name":"Nephrology, Dialysis and Transplantation Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2882-4226","authenticated-orcid":false,"given":"Francesco","family":"Pesce","sequence":"additional","affiliation":[{"name":"Department of Translational Medicine and Surgery, Universit\u00e0 Cattolica dl Sacro Cuore, 00168 Rome, Italy"},{"name":"Division of Renal Medicine, \u201cOspedale Isola Tiberina\u2014Gemelli Isola\u201d, 00186 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,6,13]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1093\/ckj\/sfx117","article-title":"A comparative analysis of survival of patients on dialysis and after kidney transplantation","volume":"11","author":"Kaballo","year":"2018","journal-title":"Clin. Kidney J."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"2093","DOI":"10.1111\/j.1600-6143.2011.03686.x","article-title":"Systematic review: Kidney transplantation compared with dialysis in clinically relevant outcomes","volume":"11","author":"Tonelli","year":"2011","journal-title":"Am. J. Transplant. Off. J. Am. Soc. Transplant. Am. Soc. Transpl. Surg."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"286","DOI":"10.2215\/CJN.15971020","article-title":"Long-Term Infectious Complications of Kidney Transplantation","volume":"17","author":"Agrawal","year":"2022","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"388","DOI":"10.1111\/j.1600-6143.2011.03840.x","article-title":"Understanding the Causes of Kidney Transplant Failure: The Dominant Role of Antibody-Mediated Rejection and Nonadherence","volume":"12","author":"Mengel","year":"2012","journal-title":"Am. J. Transplant."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"e13738","DOI":"10.1111\/ctr.13738","article-title":"The association of acute rejection vs recurrent glomerular disease with graft outcomes after kidney transplantation","volume":"33","author":"Singh","year":"2019","journal-title":"Clin. Transplant."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1111\/ajt.13423","article-title":"Changing Kidney Allograft Histology Early Posttransplant: Prognostic Implications of 1-Year Protocol Biopsies","volume":"16","author":"Cosio","year":"2016","journal-title":"Am. J. Transplant."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1016\/j.kint.2016.08.030","article-title":"Recent advances in our understanding of recurrent primary glomerulonephritis after kidney transplantation","volume":"91","author":"Cosio","year":"2017","journal-title":"Kidney Int."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/j.semnephrol.2022.01.005","article-title":"Glomerular Diseases of the Kidney Allograft: Toward a Precision Medicine Approach","volume":"42","author":"Zanoni","year":"2022","journal-title":"Semin. Nephrol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1016\/j.kint.2017.03.015","article-title":"Recurrent glomerulonephritis after kidney transplantation: Risk factors and allograft outcomes","volume":"92","author":"Allen","year":"2017","journal-title":"Kidney Int."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1730","DOI":"10.2215\/CJN.00280121","article-title":"Recurrent Glomerular Disease after Kidney Transplantation: Diagnostic and Management Dilemmas","volume":"16","author":"Uffing","year":"2021","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2363","DOI":"10.2215\/CJN.06720810","article-title":"Posttransplant recurrence of primary glomerulonephritis","volume":"5","author":"Ponticelli","year":"2010","journal-title":"Clin. J. Am. Soc. Nephrol. CJASN"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1944","DOI":"10.3389\/fimmu.2019.01944","article-title":"Recurrent and de novo Glomerulonephritis After Kidney Transplantation","volume":"10","author":"Lim","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1111\/nep.12255","article-title":"Recurrent glomerular disease after kidney transplantation: An update of selected areas and the impact of protocol biopsy","volume":"19","author":"Morozumi","year":"2014","journal-title":"Nephrol. Carlton Vic."},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Infante, B., Rossini, M., Leo, S., Troise, D., Netti, G.S., Ranieri, E., Gesualdo, L., Castellano, G., and Stallone, G. (2020). Recurrent Glomerulonephritis after Renal Transplantation: The Clinical Problem. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21175954"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1079","DOI":"10.1038\/ki.2013.377","article-title":"C3 glomerulopathy: Consensus report","volume":"84","author":"Pickering","year":"2013","journal-title":"Kidney Int."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"272","DOI":"10.1159\/000507254","article-title":"Update on C3 Glomerulopathy: A Complement-Mediated Disease","volume":"144","author":"Lucientes","year":"2020","journal-title":"Nephron"},{"key":"ref_17","first-page":"305","article-title":"Renal allograft failure due to recurrent dense intramembranous deposit disease","volume":"21","author":"Eddy","year":"1984","journal-title":"Clin. Nephrol"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"632","DOI":"10.5500\/wjt.v6.i4.632","article-title":"Complement related kidney diseases: Recurrence after transplantation","volume":"6","author":"Salvadori","year":"2016","journal-title":"World J. Transplant."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1053\/j.ajkd.2018.09.002","article-title":"Kidney Transplantation in C3 Glomerulopathy: A Case Series","volume":"73","author":"Avasare","year":"2019","journal-title":"Am. J. Kidney Dis. Off. J. Natl. Kidney Found."},{"key":"ref_20","first-page":"222","article-title":"Recurrence of immune complex and complement-mediated membranoproliferative glomerulonephritis in kidney transplantation","volume":"38","author":"Polanco","year":"2023","journal-title":"Nephrol. Dial. Transplant. Off. Publ. Eur. Dial. Transpl. Assoc\u2014Eur. Ren. Assoc."},{"key":"ref_21","first-page":"e940502-1","article-title":"Recurrent Membranoproliferative Glomerulonephritis After Kidney Transplantation: Risk Factors and Impact on Graft Survival","volume":"28","author":"Mazzali","year":"2023","journal-title":"Ann. Transplant."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"2669","DOI":"10.1016\/j.transproceed.2015.09.053","article-title":"Likely Recurrence of C3 Glomerulonephritis in Kidney Transplantation: An Entity to Bear in Mind: Case Report","volume":"47","year":"2015","journal-title":"Transplant. Proc."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1780","DOI":"10.1093\/ndt\/gfz021","article-title":"Clinical course and outcome after kidney transplantation in patients with C3 glomerulonephritis due to CFHR5 nephropathy","volume":"34","author":"Frangou","year":"2019","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_24","first-page":"2260","article-title":"C3 glomerulonephritis secondary to mutations in factors H and I: Rapid recurrence in deceased donor kidney transplant effectively treated with eculizumab","volume":"33","author":"Garg","year":"2018","journal-title":"Nephrol. Dial. Transplant. Off. Publ. Eur. Dial. Transpl. Assoc\u2014Eur. Ren. Assoc."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1053\/j.ajkd.2007.11.032","article-title":"Complement factor H deficiency and posttransplantation glomerulonephritis with isolated C3 deposits","volume":"51","author":"Boyer","year":"2008","journal-title":"Am. J. Kidney Dis. Off. J. Natl. Kidney Found."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"994","DOI":"10.2215\/CJN.16801221","article-title":"Association of Histologic Parameters with Outcome in C3 Glomerulopathy and Idiopathic Immunoglobulin-Associated Membranoproliferative Glomerulonephritis","volume":"17","author":"Barbour","year":"2022","journal-title":"Clin. J. Am. Soc. Nephrol. CJASN"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"945","DOI":"10.2215\/CJN.05490522","article-title":"Prognostication for C3 Glomerulopathy and Idiopathic Immunoglobulin-Associated Membranoproliferative Glomerulonephritis","volume":"17","author":"Praga","year":"2022","journal-title":"Clin. J. Am. Soc. Nephrol. CJASN"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1111\/ajt.14625","article-title":"The Banff 2017 Kidney Meeting Report: Revised diagnostic criteria for chronic active T cell-mediated rejection, antibody-mediated rejection, and prospects for integrative endpoints for next-generation clinical trials","volume":"18","author":"Haas","year":"2018","journal-title":"Am. J. Transplant. Off. J. Am. Soc. Transplant. Am. Soc. Transpl. Surg."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1681\/ASN.2018070777","article-title":"Archetype Analysis Identifies Distinct Profiles in Renal Transplant Recipients with Transplant Glomerulopathy Associated with Allograft Survival","volume":"30","author":"Aubert","year":"2019","journal-title":"J. Am. Soc. Nephrol. JASN"},{"key":"ref_30","first-page":"235","article-title":"Transplant glomerulopathy","volume":"31","author":"Filippone","year":"2018","journal-title":"Mod. Pathol. Off. J. U S Can. Acad. Pathol. Inc."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"582","DOI":"10.1016\/j.ekir.2019.01.018","article-title":"Glomerular C3 Deposition Is an Independent Risk Factor for Allograft Failure in Kidney Transplant Recipients With Transplant Glomerulopathy","volume":"4","author":"Panzer","year":"2019","journal-title":"Kidney Int. Rep."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1051","DOI":"10.1016\/j.pcl.2022.08.001","article-title":"Acute Postinfectious Glomerulonephritis","volume":"69","author":"Duong","year":"2022","journal-title":"Pediatr. Clin. North. Am."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"829","DOI":"10.1681\/ASN.2019080851","article-title":"Anti-Factor B Antibodies and Acute Postinfectious GN in Children","volume":"31","author":"Chauvet","year":"2020","journal-title":"J. Am. Soc. Nephrol. JASN"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1038\/ki.2012.384","article-title":"Atypical postinfectious glomerulonephritis is associated with abnormalities in the alternative pathway of complement","volume":"83","author":"Sethi","year":"2013","journal-title":"Kidney Int."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1093\/ckj\/sfw032","article-title":"Revisiting post-infectious glomerulonephritis in the emerging era of C3 glomerulopathy","volume":"9","author":"Khalighi","year":"2016","journal-title":"Clin. Kidney J."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"E6","DOI":"10.1016\/S0272-6386(00)70356-8","article-title":"Type I membranoproliferative glomerulonephritis in a renal allograft: A recurrence induced by a cytomegalovirus infection?","volume":"35","author":"Andresdottir","year":"2000","journal-title":"Am. J. Kidney Dis. Off. J. Natl. Kidney Found."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1007\/s11684-020-0754-0","article-title":"Single-cell RNA-seq data analysis on the receptor ACE2 expression reveals the potential risk of different human organs vulnerable to 2019-nCoV infection","volume":"14","author":"Zou","year":"2020","journal-title":"Front Med."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1061","DOI":"10.1001\/jama.2020.1585","article-title":"Clinical Characteristics of 138 Hospitalized Patients With 2019 Novel Coronavirus-Infected Pneumonia in Wuhan, China","volume":"323","author":"Wang","year":"2020","journal-title":"JAMA"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"m1985","DOI":"10.1136\/bmj.m1985","article-title":"Features of 20\u2009133 UK Patients in Hospital with COVID-19 Using the ISARIC WHO Clinical Characterisation Protocol: Prospective Observational Cohort Study","volume":"369","author":"Docherty","year":"2020","journal-title":"BMJ"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"997","DOI":"10.1136\/gutjnl-2020-321013","article-title":"Gastrointestinal symptoms of 95 cases with SARS-CoV-2 infection","volume":"69","author":"Lin","year":"2020","journal-title":"Gut"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"e2176","DOI":"10.1002\/rmv.2176","article-title":"COVID-19 and kidney injury: Pathophysiology and molecular mechanisms","volume":"31","author":"Ahmadian","year":"2021","journal-title":"Rev. Med. Virol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"594849","DOI":"10.3389\/fimmu.2020.594849","article-title":"Complement Activation in Kidneys of Patients With COVID-19","volume":"11","author":"Pfister","year":"2020","journal-title":"Front. Immunol."},{"key":"ref_43","doi-asserted-by":"crossref","unstructured":"Wen, J., Wang, W., Xu, F., Sun, J., Chen, J., and Ni, X. (2018). C3 glomerulonephritis and thrombotic microangiopathy of renal allograft after pulmonary infection in a male with concomitant two complement factor I gene variations: A case report. BMC Nephrol., 19.","DOI":"10.1186\/s12882-018-0952-z"},{"key":"ref_44","doi-asserted-by":"crossref","unstructured":"Lim, J.-H., Shin, S.-W., Kim, M.-S., Han, M.-H., Kim, Y.-J., Jung, H.-Y., Choi, J.-Y., Cho, J.-H., Park, S.-H., and Kim, Y.-L. (2023). Recurrent C3 Glomerulonephritis along with BK-Virus-Associated Nephropathy after Kidney Transplantation: A Case Report. Med. Kaunas. Lith., 59.","DOI":"10.3390\/medicina59071308"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1053\/j.ackd.2019.12.003","article-title":"C3 Glomerulopathy: Pathogenesis and Treatment","volume":"27","author":"Ahmad","year":"2020","journal-title":"Adv. Chronic Kidney Dis."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1287","DOI":"10.2215\/CJN.15241219","article-title":"Mycophenolate Mofetil in C3 Glomerulopathy and Pathogenic Drivers of the Disease","volume":"15","author":"Lucientes","year":"2020","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1975","DOI":"10.1007\/s00467-013-2503-y","article-title":"Eculizumab and recurrent C3 glomerulonephritis","volume":"28","author":"Gurkan","year":"2013","journal-title":"Pediatr. Nephrol."},{"key":"ref_48","doi-asserted-by":"crossref","unstructured":"Gonzalez Suarez, M.L., Thongprayoon, C., Hansrivijit, P., Kovvuru, K., Kanduri, S.R., Aeddula, N.R., Pivovarova, A.I., Chewcharat, A., Bathini, T., and Mao, M.A. (2020). Treatment of C3 Glomerulopathy in Adult Kidney Transplant Recipients: A Systematic Review. Med. Sci. Basel Switz., 8.","DOI":"10.3390\/medsci8040044"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1233","DOI":"10.1056\/NEJMoa061648","article-title":"The complement inhibitor eculizumab in paroxysmal nocturnal hemoglobinuria","volume":"355","author":"Hillmen","year":"2006","journal-title":"N. Engl. J. Med."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1016\/S0140-6736(17)30062-4","article-title":"Haemolytic uraemic syndrome","volume":"390","author":"Fakhouri","year":"2017","journal-title":"Lancet Lond. Engl."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"412","DOI":"10.23876\/j.krcp.21.248","article-title":"Application of C5 inhibitors in glomerular diseases in 2021","volume":"41","author":"Werion","year":"2022","journal-title":"Kidney Res. Clin. Pract."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"4197","DOI":"10.1007\/s00467-023-06035-4","article-title":"Successful treatment with avacopan [CCX168] in a pediatric patient with C3 glomerulonephritis","volume":"38","author":"Zotta","year":"2023","journal-title":"Pediatr. Nephrol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"2150","DOI":"10.1016\/j.ekir.2022.07.004","article-title":"Alternative Complement Pathway Inhibition With Iptacopan for the Treatment of C3 Glomerulopathy-Study Design of the APPEAR-C3G Trial","volume":"7","author":"Bomback","year":"2022","journal-title":"Kidney Int. Rep."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1111\/ajt.13042","article-title":"Advances in the understanding of complement mediated glomerular disease: Implications for recurrence in the transplant setting","volume":"15","author":"Barbour","year":"2015","journal-title":"Am. J. Transplant. Off. J. Am. Soc. Transplant. Am. Soc. Transpl. Surg."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"721","DOI":"10.1038\/ki.2010.1","article-title":"Recurrent membranoproliferative glomerulonephritis after kidney transplantation","volume":"77","author":"Lorenz","year":"2010","journal-title":"Kidney Int."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1016\/j.clim.2017.10.013","article-title":"Recurrent allograft C3 glomerulonephritis and unsuccessful eculizumab treatment","volume":"187","author":"Kaartinen","year":"2018","journal-title":"Clin. Immunol. Orlando Fla."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1159\/000520387","article-title":"Repository Corticotropin in Treating de novo C3 Glomerulonephritis after Transplantation","volume":"2","author":"Naseer","year":"2022","journal-title":"Glomerular Dis."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"1290","DOI":"10.1681\/ASN.2009101025","article-title":"Melanocortin 1 receptor agonists reduce proteinuria","volume":"21","author":"Lindskog","year":"2010","journal-title":"J. Am. Soc. Nephrol. JASN"},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1038\/nrneph.2011.190","article-title":"The renaissance of corticotropin therapy in proteinuric nephropathies","volume":"8","author":"Gong","year":"2011","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"1287","DOI":"10.1016\/j.kint.2020.01.035","article-title":"The long-acting C5 inhibitor, Ravulizumab, is effective and safe in adult patients with atypical hemolytic uremic syndrome na\u00efve to complement inhibitor treatment","volume":"97","author":"Rondeau","year":"2020","journal-title":"Kidney Int."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"1106","DOI":"10.1016\/j.kint.2020.03.011","article-title":"Is ravulizumab the new treatment of choice for atypical hemolytic uremic syndrome [aHUS]?","volume":"97","author":"Menne","year":"2020","journal-title":"Kidney Int."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"1603","DOI":"10.1016\/j.ekir.2021.03.884","article-title":"Long-Term Efficacy and Safety of the Long-Acting Complement C5 Inhibitor Ravulizumab for the Treatment of Atypical Hemolytic Uremic Syndrome in Adults","volume":"6","author":"Barbour","year":"2021","journal-title":"Kidney Int. Rep."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1016\/j.kint.2020.10.046","article-title":"The long-acting C5 inhibitor, ravulizumab, is effective and safe in pediatric patients with atypical hemolytic uremic syndrome na\u00efve to complement inhibitor treatment","volume":"100","author":"Ariceta","year":"2021","journal-title":"Kidney Int."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1038\/ng912","article-title":"Uncontrolled C3 activation causes membranoproliferative glomerulonephritis in mice deficient in complement factor, H","volume":"31","author":"Pickering","year":"2002","journal-title":"Nat. Genet."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"9649","DOI":"10.1073\/pnas.0601094103","article-title":"Prevention of C5 activation ameliorates spontaneous and experimental glomerulonephritis in factor H-deficient mice","volume":"103","author":"Pickering","year":"2006","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1111\/j.1523-1755.2004.00365.x","article-title":"Proliferative glomerulonephritis with monoclonal IgG deposits: A distinct entity mimicking immune-complex glomerulonephritis","volume":"65","author":"Nasr","year":"2004","journal-title":"Kidney Int."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"4292","DOI":"10.1182\/blood-2012-07-445304","article-title":"Monoclonal gammopathy of renal significance: When MGUS is no longer undetermined or insignificant","volume":"120","author":"Leung","year":"2012","journal-title":"Blood"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1038\/ki.2014.408","article-title":"Diagnosis of monoclonal gammopathy of renal significance","volume":"87","author":"Bridoux","year":"2015","journal-title":"Kidney Int."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1053\/j.ajkd.2013.02.370","article-title":"C3 glomerulonephritis associated with monoclonal gammopathy: A case series","volume":"62","author":"Zand","year":"2013","journal-title":"Am. J. Kidney Dis. Off. J. Natl. Kidney Found."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1084\/jem.175.4.939","article-title":"Activation of the alternative pathway of complement by monoclonal lambda light chains in membranoproliferative glomerulonephritis","volume":"175","author":"Meri","year":"1992","journal-title":"J. Exp. Med."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"2260","DOI":"10.3389\/fimmu.2018.02260","article-title":"Both Monoclonal and Polyclonal Immunoglobulin Contingents Mediate Complement Activation in Monoclonal Gammopathy Associated-C3 Glomerulopathy","volume":"9","author":"Chauvet","year":"2018","journal-title":"Front. Immunol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"1284","DOI":"10.1016\/j.mayocp.2013.08.002","article-title":"Monoclonal gammopathy-associated proliferative glomerulonephritis","volume":"88","author":"Sethi","year":"2013","journal-title":"Mayo Clin. Proc."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1053\/j.ajkd.2021.04.015","article-title":"Kidney Transplantation in Patients With Monoclonal Gammopathy of Renal Significance [MGRS] -Associated Lesions: A Case Series","volume":"79","author":"Heybeli","year":"2022","journal-title":"Am. J. Kidney Dis. Off. J. Natl. Kidney Found."},{"key":"ref_74","first-page":"e939726","article-title":"A 78-Year-Old Man with Chronic Kidney Disease and Monoclonal Gammopathy Who Developed Post-Transplant C3 Glomerulopathy\u2014Recurrence or De Novo? A Case Report and Literature Review","volume":"24","year":"2023","journal-title":"Am. J. Case Rep."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1016\/j.kint.2018.01.037","article-title":"C3 glomerulopathy associated with monoclonal Ig is a distinct subtype","volume":"94","author":"Ravindran","year":"2018","journal-title":"Kidney Int."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"794","DOI":"10.1093\/ckj\/sfw090","article-title":"C3 glomerulopathy in adults: A distinct patient subset showing frequent association with monoclonal gammopathy and poor renal outcome","volume":"9","author":"Lloyd","year":"2016","journal-title":"Clin. Kidney J."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1016\/j.kint.2018.01.028","article-title":"Proliferative glomerulonephritis with monoclonal immunoglobulin G deposits is associated with high rate of early recurrence in the allograft","volume":"94","author":"Said","year":"2018","journal-title":"Kidney Int."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1053\/j.ajkd.2011.05.003","article-title":"Proliferative glomerulonephritis with monoclonal IgG deposits recurs or may develop de novo in kidney allografts","volume":"58","author":"Albawardi","year":"2011","journal-title":"Am. J. Kidney Dis. Off. J. Natl. Kidney Found."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1111\/nep.13280","article-title":"A case of recurrent proliferative glomerulonephritis with monoclonal IgG deposits or de novo C3 glomerulonephritis after kidney transplantation","volume":"23","author":"Tamura","year":"2018","journal-title":"Nephrol. Carlton Vic."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1016\/j.nefro.2018.03.008","article-title":"Three cases of monoclonal gammopathy of renal significance after kidney transplantation. De novo C3 glomerulopathy","volume":"39","author":"Serra","year":"2019","journal-title":"Nefrologia"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"122","DOI":"10.2215\/CJN.05750710","article-title":"Proliferative Glomerulonephritis with Monoclonal IgG Deposits Recurs in the Allograft","volume":"6","author":"Nasr","year":"2011","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"1437","DOI":"10.1182\/blood-2016-08-737163","article-title":"Treatment of B-cell disorder improves renal outcome of patients with monoclonal gammopathy-associated C3 glomerulopathy","volume":"129","author":"Chauvet","year":"2017","journal-title":"Blood"},{"key":"ref_83","doi-asserted-by":"crossref","unstructured":"Moog, P., Jost, P.J., and B\u00fcttner-Herold, M. (2018). Eculizumab as salvage therapy for recurrent monoclonal gammopathy-induced C3 glomerulopathy in a kidney allograft. BMC Nephrol., 19.","DOI":"10.1186\/s12882-018-0904-7"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"1609","DOI":"10.2215\/CJN.10611110","article-title":"Patterns of noncryoglobulinemic glomerulonephritis with monoclonal Ig deposits: Correlation with IgG subclass and response to rituximab","volume":"6","author":"Guiard","year":"2011","journal-title":"Clin. J. Am. Soc. Nephrol. CJASN"},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"2180508","DOI":"10.1155\/2017\/2180508","article-title":"Rituximab for Treatment of Membranoproliferative Glomerulonephritis and C3 Glomerulopathies","volume":"2017","author":"Rudnicki","year":"2017","journal-title":"BioMed Res. Int."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1159\/000521901","article-title":"Use of Rituximab as an Off-Label Medication in Glomerular Diseases: Clinical Perspective","volume":"31","author":"AlSahow","year":"2022","journal-title":"Med. Princ. Pract."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"866","DOI":"10.1053\/j.ajkd.2019.01.031","article-title":"Management of Kidney Transplant Recipients by General Nephrologists: Core Curriculum 2019","volume":"73","author":"Voora","year":"2019","journal-title":"Am. J. Kidney Dis. Off. J. Natl. Kidney Found."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"100839","DOI":"10.1016\/j.trre.2024.100839","article-title":"Recurrent C3 glomerulopathy after kidney transplantation","volume":"38","author":"Obata","year":"2024","journal-title":"P. Transplant. Rev."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"1289812","DOI":"10.3389\/fmed.2023.1289812","article-title":"C3 glomerulopathies: Dense deposit disease and C3 glomerulonephritis","volume":"10","author":"Ponticelli","year":"2023","journal-title":"Front. Med."},{"key":"ref_90","first-page":"569","article-title":"C3 Glomerulopathy: Novel Treatment Paradigms","volume":"9","author":"Bomback","year":"2023","journal-title":"Kidney Int. Rep."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/25\/12\/6508\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T14:57:47Z","timestamp":1760108267000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/25\/12\/6508"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,6,13]]},"references-count":90,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2024,6]]}},"alternative-id":["ijms25126508"],"URL":"https:\/\/doi.org\/10.3390\/ijms25126508","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,6,13]]}}}