{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T15:56:24Z","timestamp":1792425384600,"version":"4.5.0"},"reference-count":81,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2020,1,14]],"date-time":"2020-01-14T00:00:00Z","timestamp":1578960000000},"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>In April 2012, a group of nephrologists organized a consensus conference in Cambridge (UK) on type II membranoproliferative glomerulonephritis and decided to use a new terminology, \u201cC3 glomerulopathy\u201d (C3 GP). Further knowledge on the complement system and on kidney biopsy contributed toward distinguishing this disease into three subgroups: dense deposit disease (DDD), C3 glomerulonephritis (C3 GN), and the CFHR5 nephropathy. The persistent presence of microhematuria with or without light or heavy proteinuria after an infection episode suggests the potential onset of C3 GP. These nephritides are characterized by abnormal activation of the complement alternative pathway, abnormal deposition of C3 in the glomeruli, and progression of renal damage to end-stage kidney disease. The diagnosis is based on studying the complement system, relative genetics, and kidney biopsies. The treatment gap derives from the absence of a robust understanding of their natural outcome. Therefore, a specific treatment for the different types of C3 GP has not been established. Recommendations have been obtained from case series and observational studies because no randomized clinical trials have been conducted. Current treatment is based on corticosteroids and antiproliferative drugs (cyclophosphamide, mycophenolate mofetil), monoclonal antibodies (rituximab) or complement inhibitors (eculizumab). In some cases, it is suggested to include sessions of plasma exchange.<\/jats:p>","DOI":"10.3390\/ijms21020525","type":"journal-article","created":{"date-parts":[[2020,1,15]],"date-time":"2020-01-15T10:30:27Z","timestamp":1579084227000},"page":"525","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":41,"title":["A Narrative Review on C3 Glomerulopathy: A Rare Renal Disease"],"prefix":"10.3390","volume":"21","author":[{"given":"Francesco Paolo","family":"Schena","sequence":"first","affiliation":[{"name":"Department of Emergency and Organ Transplantation, Renal Unit, University of Bari, 70124 Bari, Italy"},{"name":"Schena Foundation, European Center for the Study of Renal Diseases, 70010 Valenzano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0834-5586","authenticated-orcid":false,"given":"Pasquale","family":"Esposito","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Division of Nephrology, Dialysis and Transplantation, University of Genoa and IRCCS Ospedale Policlinico San Martino, 16132 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michele","family":"Rossini","sequence":"additional","affiliation":[{"name":"Department of Emergency and Organ Transplantation, Renal Unit, University of Bari, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,1,14]]},"reference":[{"key":"ref_1","unstructured":"Schena, F.P., and Alpers, G.E. (2015). Membranoproliferative glomerulonephritis and cryoglobulinemic glomerulonephritis. Comprehensive Clinical Nephrology, Elsevier. [5th ed.]."},{"key":"ref_2","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_3","doi-asserted-by":"crossref","first-page":"494","DOI":"10.1038\/nrneph.2010.85","article-title":"C3 glomerulopathy: A new classification","volume":"6","author":"Fakhouri","year":"2010","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1681\/ASN.2017111183","article-title":"Clusters Not Classifications: Making Sense of Complement-Mediated Kidney Injury","volume":"29","author":"Cook","year":"2019","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1038\/nrneph.2017.156","article-title":"The renaissance of complement therapeutics","volume":"14","author":"Ricklin","year":"2018","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"4590","DOI":"10.4049\/jimmunol.163.8.4590","article-title":"Nephritogenic lambda light chain dimer: A unique human miniautoantibody against complement factor H","volume":"163","author":"Jokiranta","year":"1999","journal-title":"J Immunol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1016\/j.molimm.2012.05.009","article-title":"Factor H autoantibodies in membranoproliferative glomerulonephritis","volume":"52","author":"Goodship","year":"2012","journal-title":"Mol. Immunol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1093\/ckj\/sfs002","article-title":"Anti-factor H antibody affecting factor H cofactor activity in a patient with dense deposit disease","volume":"5","author":"Nozal","year":"2012","journal-title":"Clin. Kidney J."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"5129","DOI":"10.4049\/jimmunol.1402770","article-title":"Anti-factor H autoantibodies in C3 glomerulopathies and in atypical hemolytic uremic syndrome: One target, two diseases","volume":"194","author":"Blanc","year":"2015","journal-title":"J. Immunol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1055\/s-0034-1376334","article-title":"C3 glomerulopathy: The genetic and clinical findings in dense deposit disease and C3 glomerulonephritis","volume":"40","author":"Xiao","year":"2014","journal-title":"Semin. Thromb. Hemost."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1016\/j.kint.2017.07.015","article-title":"Complete functional characterization of disease-associated genetic variants in the complement factor H gene","volume":"93","author":"Merinero","year":"2018","journal-title":"Kidney Int."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1093\/ndt\/gfu317","article-title":"Update on C3 glomerulopathy","volume":"31","author":"Barbour","year":"2016","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1126\/science.164.3878.436","article-title":"Serum C\u20193 lytic system in patients with glomerulonephritis","volume":"164","author":"Spitzer","year":"1969","journal-title":"Science"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"764","DOI":"10.4049\/jimmunol.113.3.764","article-title":"Lysis of human erythrocytes due to activation of the alternate complement pathway by nephritic factor (C3NeF)","volume":"113","author":"Arroyave","year":"1974","journal-title":"J. Immunol."},{"key":"ref_15","first-page":"789","article-title":"Formation in the presence of C3 nephritic factor (C3NeF) of an alternative pathway C3 convertase containing uncleaved B","volume":"31","author":"Daha","year":"1976","journal-title":"Immunology"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"812","DOI":"10.4049\/jimmunol.119.3.812","article-title":"The incorporation of C3 nephritic factor (C3NeF) into a stabilized C3 convertase, C3bBb (C3NeF), and its release after decay of convertase function","volume":"119","author":"Daha","year":"1977","journal-title":"J. Immunol."},{"key":"ref_17","first-page":"425","article-title":"Identification of nephritic factor as an immunoglobulin","volume":"33","author":"Williams","year":"1978","journal-title":"Clin. Exp. Immunol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/j.imlet.2014.01.014","article-title":"Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome","volume":"160","author":"Reuter","year":"2014","journal-title":"Immunol. Lett."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1232","DOI":"10.1016\/j.kint.2017.04.017","article-title":"C5 nephritic factors drive the biological phenotype of C3 glomerulopathies","volume":"92","author":"Marinozzi","year":"2017","journal-title":"Kidney Int."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1249","DOI":"10.1172\/JCI109787","article-title":"Nephritic factor of the classical pathway of complement: Immunoglobulin G autoantibody directed against the classical pathway C3 convetase enzyme","volume":"65","author":"Halbwachs","year":"1980","journal-title":"J. Clin. Investig."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1016\/0090-1229(90)90144-F","article-title":"Two types of C3 nephritic factor: Properdin-dependent C3NeF and properdin-independent C3NeF","volume":"56","author":"Tanuma","year":"1990","journal-title":"Clin. Immunol. Immunopathol."},{"key":"ref_22","first-page":"240","article-title":"Biological significance of the C3 nephritic factor in membranoproliferative glomerulonephritis","volume":"18","author":"Schena","year":"1982","journal-title":"Clin. Nephrol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"991","DOI":"10.1016\/j.mayocp.2018.05.019","article-title":"C3 Glomerulopathy: Ten Years\u2019 Experience at Mayo Clinic","volume":"93","author":"Ravindran","year":"2018","journal-title":"Mayo Clin. Proc."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"2329","DOI":"10.3389\/fimmu.2018.02329","article-title":"Unraveling the Molecular Mechanisms Underlying Complement Dysregulation by Nephritic Factors in C3G and IC-MPGN","volume":"9","author":"Donadelli","year":"2018","journal-title":"Front. Immunol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"46","DOI":"10.2215\/CJN.04700513","article-title":"C3 glomerulopathy: Clinicopathologic features and predictors of outcome","volume":"9","author":"Traynor","year":"2014","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1476","DOI":"10.1016\/j.molimm.2010.02.002","article-title":"Anti-factor B autoantibody in dense deposit disease","volume":"47","author":"Strobel","year":"2010","journal-title":"Mol. Immunol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"2340","DOI":"10.1056\/NEJMc1107484","article-title":"Combined C3b and factor B autoantibodies and MPGN type II","volume":"365","author":"Chen","year":"2011","journal-title":"N Engl. J. Med."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"2447","DOI":"10.1681\/ASN.2007030356","article-title":"Dense Deposit Disease Focus Group. New approaches to the treatment of dense deposit disease","volume":"18","author":"Smith","year":"2007","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"977","DOI":"10.1016\/j.kint.2017.10.022","article-title":"C3 glomerulonephritis and dense deposit disease share a similar disease course in a large United States cohort of patients with C3 glomerulopathy","volume":"93","author":"Bomback","year":"2018","journal-title":"Kidney Int."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1038\/s41581-018-0107-2","article-title":"C3 glomerulopathy\u2014Understanding a rare complement-driven renal disease","volume":"15","author":"Smith","year":"2019","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1436","DOI":"10.2215\/CJN.09541010","article-title":"Familial C3 glomerulopathy associated with CFHR5 mutations: Clinical characteristics of 91 patients in 16 pedigrees","volume":"6","author":"Athanasiou","year":"2011","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1038\/ki.2012.63","article-title":"Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies","volume":"82","author":"Servais","year":"2012","journal-title":"Kidney Int."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"1153","DOI":"10.1038\/ki.2015.227","article-title":"Spanish Group for the Study of Glomerular Diseases (GLOSEN). Effectiveness of mycophenolate mofetil in C3 glomerulonephritis","volume":"88","author":"Rabasco","year":"2015","journal-title":"Kidney Int."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1016\/j.molimm.2016.01.010","article-title":"Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal outcome","volume":"71","author":"Iatropoulos","year":"2016","journal-title":"Mol. Immunol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1159\/000479012","article-title":"Immunosuppressive Treatment in C3 Glomerulopathy: Is it Really Effective?","volume":"46","author":"Caliskan","year":"2017","journal-title":"Am. J. Nephrol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1053\/j.ajkd.2017.11.019","article-title":"Patterns of Clinical Response to Eculizumab in Patients with C3 Glomerulopathy","volume":"72","author":"Lapeyraque","year":"2018","journal-title":"Am. J. Kidney Dis."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"794","DOI":"10.1016\/S0140-6736(10)60670-8","article-title":"Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with glomerulonephritis","volume":"376","author":"Gale","year":"2010","journal-title":"Lancet"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"3702","DOI":"10.1172\/JCI43343","article-title":"Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation","volume":"120","author":"Heurich","year":"2010","journal-title":"J. Clin. Investig."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"1155","DOI":"10.1681\/ASN.2012020166","article-title":"A hybrid CFHR3-1 gene causes familial C3 glomerulopathy","volume":"23","author":"Malik","year":"2012","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"2434","DOI":"10.1172\/JCI68280","article-title":"C3 glomerulopathy associated CFHR1 mutation alters FHR oligomerization and complement regulation","volume":"123","author":"Tortajada","year":"2013","journal-title":"J. Clin. Investig."},{"key":"ref_41","first-page":"426","article-title":"Progressive IgA Nephropathy is Associated with low circulating mannan-binding lectin-associated serine protease-3 (MASP-3) and increased glomerular factor H-related protein-5 (FHR5) Deposition","volume":"3","author":"Troldborg","year":"2017","journal-title":"Kidney Int. Rep."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1038\/ki.2013.340","article-title":"Toward a working definition of C3 glomerulopathy by immunofluorescence","volume":"85","author":"Hou","year":"2014","journal-title":"Kidney Int."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"2852","DOI":"10.1681\/ASN.2014040406","article-title":"C4d as a diagnostic tool in proliferative GN","volume":"26","author":"Sethi","year":"2015","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1016\/j.ekir.2019.02.006","article-title":"Glomerular C4d staining does not exclude a C3 Glomerulopathy","volume":"4","author":"Singh","year":"2019","journal-title":"Kidney Int. Rep."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1038\/s41581-018-0102-7","article-title":"The evaluation of monoclonal gammopathy of renal significance: A consensus report of the International Kidney and Monoclonal Gammopathy Research Group","volume":"15","author":"Leung","year":"2019","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_46","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."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1016\/j.kint.2015.12.003","article-title":"Diagnosis of complement alternative pathway disorders","volume":"89","author":"Angioi","year":"2016","journal-title":"Kidney Int."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1016\/j.molimm.2014.06.030","article-title":"Are complement deficiencies really rare? Overview on prevalence, clinical importance and modern diagnostic approach","volume":"61","author":"Grumach","year":"2014","journal-title":"Mol. Immunol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1876","DOI":"10.2215\/CJN.01820214","article-title":"Defining the complement biomarker profile of C3 glomerulopathy","volume":"9","author":"Zhang","year":"2014","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"1829","DOI":"10.2215\/CJN.09620914","article-title":"Circulating complement levels and C3 glomerulopathy","volume":"9","author":"Fervenza","year":"2014","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"886","DOI":"10.3389\/fimmu.2019.00886","article-title":"Nephritic Factors: An Overview of Classification, Diagnostic Tools and Clinical Associations","volume":"10","author":"Corvillo","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"265","DOI":"10.2215\/CJN.07900811","article-title":"Causes of alternative pathway dysregulation in dense deposit disease","volume":"7","author":"Zhang","year":"2012","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1111\/j.1365-2249.1994.tb06530.x","article-title":"Occurrence of C3 nephritic factor and C4 nephritic factor in membranoproliferative glomerulonephritis (MPGN)","volume":"95","author":"Ohi","year":"1994","journal-title":"Clin. Exp. Immunol."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/j.imbio.2013.06.004","article-title":"Standardisation of the factor H autoantibody assay","volume":"219","author":"Watson","year":"2014","journal-title":"Immunobiology"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"2464","DOI":"10.4049\/jimmunol.1701695","article-title":"Statistical Validation of Rare Complement Variants Provides Insights into the Molecular Basis of Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy","volume":"200","author":"Osborne","year":"2018","journal-title":"J. Immunol."},{"key":"ref_56","first-page":"1607","article-title":"Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family","volume":"12","author":"Pouw","year":"2018","journal-title":"Front. Immunol."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1111\/j.1600-6143.2010.03333.x","article-title":"Recurrence of complement factor H-related protein 5 nephropathy in a renal transplant","volume":"11","author":"Vernon","year":"2011","journal-title":"Am. J. Transplant."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"2097","DOI":"10.1007\/s00467-009-1137-6","article-title":"Disease recurrence in paediatric renal transplantation","volume":"11","author":"Cochat","year":"2009","journal-title":"Pediatr. Nephrol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1093\/ckj\/sfw020","article-title":"Kidney transplant outcomes in familial C3 glomerulopathy","volume":"3","author":"Wong","year":"2016","journal-title":"Clin. Kidney J."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"748","DOI":"10.2215\/CJN.12901211","article-title":"Eculizumab for dense deposit disease and C3 glomerulonephritis","volume":"7","author":"Bomback","year":"2012","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"203","DOI":"10.5500\/wjt.v8.i6.203","article-title":"Complement-mediated renal diseases after kidney transplantation\u2014Current diagnostic and therapeutic options in de novo and recurrent diseases","volume":"8","author":"Abbas","year":"2018","journal-title":"World J. Transplant."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"2055","DOI":"10.1007\/s00467-014-2839-y","article-title":"Eculizumab in dense-deposit disease after renal transplantation","volume":"29","author":"Cabrera","year":"2014","journal-title":"Pediatr. Nephrol."},{"key":"ref_63","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_64","doi-asserted-by":"crossref","unstructured":"Uffing, A., P\u00e9rez-S\u00e1ez, M.J., La Manna, G., Comai, G., Fischman, C., Farouk, S., Manfro, R.C., Bauer, A.C., Lichtenfels, B., and Mansur, J.B. (2018). A large, international study on post-transplant glomerular diseases: The TANGO project. BMC Nephrol., 19.","DOI":"10.1186\/s12882-018-1025-z"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"2169","DOI":"10.1056\/NEJMoa1208981","article-title":"Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome","volume":"368","author":"Legendre","year":"2013","journal-title":"N. Engl. J. Med."},{"key":"ref_66","doi-asserted-by":"crossref","unstructured":"Welte, T., Arnold, F., Kappes, J., Seidl, M., H\u00e4ffner, K., Bergmann, C., Walz, G., and Neumann-Haefelin, E. (2018). Treating C3 glomerulopathy with eculizumab. BMC Nephrol., 19.","DOI":"10.1186\/s12882-017-0802-4"},{"key":"ref_67","doi-asserted-by":"crossref","unstructured":"Von Visger, J., Cassol, C., Nori, U., Franco-Ahumada, G., Nadasdy, T., and Satoskar, A.A. (2019). Complete biopsy-proven resolution of deposits in recurrent proliferative glomerulonephritis with monoclonal IgG deposits (PGNMIGD) following rituximab treatment in renal allograft. BMC Nephrol., 20.","DOI":"10.1186\/s12882-019-1239-8"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"1046","DOI":"10.1111\/j.1600-6143.2011.03923.x","article-title":"Recurrent dense deposit disease after renal transplantation: An emerging role for complementary therapies","volume":"12","author":"McCaughan","year":"2012","journal-title":"Am. J. Transplant."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"1110","DOI":"10.1681\/ASN.2013070715","article-title":"Clinical findings, pathology, and outcomes of C3GN after kidney transplantation","volume":"25","author":"Zand","year":"2014","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_70","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_71","first-page":"10","article-title":"Diagnosis and treatment of C3 glomerulopathy in a center of expertise","volume":"77","author":"Koopman","year":"2019","journal-title":"Neth. J. Med."},{"key":"ref_72","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_73","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_74","doi-asserted-by":"crossref","first-page":"977","DOI":"10.1053\/j.ajkd.2010.06.021","article-title":"Dense deposit disease associated with monoclonal gammopathy of undetermined significance","volume":"56","author":"Sethi","year":"2010","journal-title":"Am. J. Kidney Dis."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"993","DOI":"10.1016\/j.imbio.2015.04.001","article-title":"Compstatin analog Cp40 inhibits complement dysregulation in vitro in C3 glomerulopathy","volume":"220","author":"Zhang","year":"2015","journal-title":"Immunobiology"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"4844","DOI":"10.4049\/jimmunol.1303131","article-title":"A humanized antibody that regulates the alternative pathway convertase: Potential for therapy of renal disease associated with nephritic factors","volume":"192","author":"Torreira","year":"2014","journal-title":"J. Immunol."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"5567","DOI":"10.4049\/jimmunol.1402013","article-title":"A novel antibody against human factor B that blocks formation of the C3bB proconvertase and inhibits complement activation in disease models","volume":"193","author":"Tortajada","year":"2014","journal-title":"J. Immunol."},{"key":"ref_78","doi-asserted-by":"crossref","unstructured":"Pauly, D., Nagel, B.M., Reinders, J., Killian, T., Wulf, M., Ackerman, S., Ehrenstein, B., Zipfel, P.F., Skerka, C., and Weber, B.H. (2014). A novel antibody against human properdin inhibits the alternative complement system and specifically detects properdin from blood samples. PLoS ONE, 9.","DOI":"10.1371\/journal.pone.0096371"},{"key":"ref_79","first-page":"4","article-title":"Acute renal failure in dense deposit disease: Complete recovery after combination therapy with immunosuppressant and plasma exchange","volume":"75","author":"Krmar","year":"2011","journal-title":"Clin. Nephrol."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"1951","DOI":"10.1007\/s00467-015-3111-9","article-title":"Successful therapy of C3Nef-positive C3 glomerulopathy with plasma therapy and immunosuppression","volume":"30","author":"Michelfelder","year":"2015","journal-title":"Pediatr. Nephrol."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1002\/jca.10026","article-title":"Management of membranoproliferative glomerulonephritis type II with plasmapheresis","volume":"17","author":"Kurtz","year":"2002","journal-title":"J. Clin. 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