{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T10:41:53Z","timestamp":1790764913733,"version":"4.1.0"},"reference-count":33,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["CJASN"],"published-print":{"date-parts":[[2018,3]]},"abstract":"<jats:sec>\n                    <jats:title>Background and objectives<\/jats:title>\n                    <jats:p>C3 glomerulopathy is a form of complement-mediated GN. Immunosuppressive therapy may be beneficial in the treatment of C3 glomerulopathy. Mycophenolate mofetil is an attractive treatment option given its role in the treatment of other complement-mediated diseases and the results of the Spanish Group for the Study of Glomerular Diseases C3 Study. Here, we study the outcomes of patients with C3 glomerulopathy treated with steroids and mycophenolate mofetil.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Design, setting, participants, &amp; measurements<\/jats:title>\n                    <jats:p>We conducted a retrospective chart review of patients in the C3 glomerulopathy registry at Columbia University and identified patients treated with mycophenolate mofetil for at least 3 months and follow-up for at least 1 year. We studied clinical, histologic, and genetic data for the whole group and compared data for those who achieved complete or partial remission (responders) with those who did not achieve remission (nonresponders). We compared remission with mycophenolate mofetil with remission with other immunosuppressive regimens.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>We identified 30 patients who met inclusion criteria. Median age was 25 years old (interquartile range, 18\u201336), median creatinine was 1.07 mg\/dl (interquartile range, 0.79\u20131.69), and median proteinuria was 3200 mg\/g creatinine (interquartile range, 1720\u20136759). The median follow-up time was 32 months (interquartile range, 21\u201368). Twenty (67%) patients were classified as responders. There were no significant differences in baseline characteristics between responders and nonresponders, although initial proteinuria was lower (median 2468 mg\/g creatinine) in responders compared with nonresponders (median 5000 mg\/g creatinine) and soluble membrane attack complex levels were higher in responders compared with nonresponders. For those tapered off mycophenolate mofetil, relapse rate was 50%. Genome-wide analysis on complement genes was done, and in 12 patients, we found 18 variants predicted to be damaging. None of these variants were previously reported to be pathogenic. Mycophenolate mofetil with steroids outperformed other immunosuppressive regimens.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Among patients who tolerated mycophenolate mofetil, combination therapy with steroids induced remission in 67% of this cohort. Heavier proteinuria at the start of therapy and lower soluble membrane attack complex levels were associated with treatment resistance.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.2215\/cjn.09080817","type":"journal-article","created":{"date-parts":[[2018,1,11]],"date-time":"2018-01-11T20:40:49Z","timestamp":1515703249000},"page":"406-413","source":"Crossref","is-referenced-by-count":97,"title":["Mycophenolate Mofetil in Combination with Steroids for Treatment of C3 Glomerulopathy"],"prefix":"10.2215","volume":"13","author":[{"given":"Rupali S.","family":"Avasare","sequence":"first","affiliation":[{"name":"Division of Nephrology, Department of Medicine, Oregon Health Science University, Portland, Oregon;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pietro A.","family":"Canetta","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Medicine, Columbia University Medical Center, New York, New York; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew S.","family":"Bomback","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Medicine, Columbia University Medical Center, New York, New York; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0712-2329","authenticated-orcid":false,"given":"Maddalena","family":"Marasa","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Medicine, Columbia University Medical Center, New York, New York; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasar","family":"Caliskan","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Internal Medicine and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasemin","family":"Ozluk","sequence":"additional","affiliation":[{"name":"Department of Pathology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yifu","family":"Li","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Medicine, Columbia University Medical Center, New York, New York; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ali G.","family":"Gharavi","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Medicine, Columbia University Medical Center, New York, New York; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerald B.","family":"Appel","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Medicine, Columbia University Medical Center, New York, New York; and"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2018,1,11]]},"reference":[{"key":"B1-20230813","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":"Mart\u00ednez-Barricarte","year":"2010","journal-title":"J Clin Invest"},{"key":"B2-20230813","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":"B3-20230813","doi-asserted-by":"crossref","first-page":"582","DOI":"10.1136\/jmg.2005.038315","article-title":"Variations in the complement regulatory genes factor H (CFH) and factor H related 5 (CFHR5) are associated with membranoproliferative glomerulonephritis type II (dense deposit disease).","volume":"43","author":"Abrera-Abeleda","year":"2006","journal-title":"J Med Genet"},{"key":"B4-20230813","doi-asserted-by":"crossref","first-page":"2150","DOI":"10.1681\/ASN.2005010103","article-title":"Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome.","volume":"16","author":"Kavanagh","year":"2005","journal-title":"J Am Soc Nephrol"},{"key":"B5-20230813","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1038\/ki.2009.472","article-title":"Mutations in components of complement influence the outcome of factor I-associated atypical hemolytic uremic syndrome.","volume":"77","author":"Bienaime","year":"2010","journal-title":"Kidney Int"},{"key":"B6-20230813","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":"B7-20230813","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":"B8-20230813","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":"B9-20230813","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 Invest"},{"key":"B10-20230813","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":"B11-20230813","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1681\/ASN.2004050380","article-title":"Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome.","volume":"16","author":"Dragon-Durey","year":"2005","journal-title":"J Am Soc Nephrol"},{"key":"B12-20230813","doi-asserted-by":"crossref","first-page":"1","DOI":"10.4049\/jimmunol.116.1.1","article-title":"C3 nephritic factor (C3NeF): Stabilization of fluid phase and cell-bound alternative pathway convertase.","volume":"116","author":"Daha","year":"1976","journal-title":"J Immunol"},{"key":"B13-20230813","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":"Medjeral-Thomas","year":"2014","journal-title":"Clin J Am Soc Nephrol"},{"key":"B14-20230813","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":"B15-20230813","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":"B16-20230813","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1038\/modpathol.3800773","article-title":"Dense deposit disease is not a membranoproliferative glomerulonephritis.","volume":"20","author":"Walker","year":"2007","journal-title":"Mod Pathol"},{"key":"B17-20230813","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":"B18-20230813","doi-asserted-by":"crossref","first-page":"1153","DOI":"10.1038\/ki.2015.227","article-title":"Effectiveness of mycophenolate mofetil in C3 glomerulonephritis.","volume":"88","author":"Rabasco","year":"2015","journal-title":"Kidney Int"},{"key":"B19-20230813","doi-asserted-by":"crossref","first-page":"57","DOI":"10.5414\/CN107945","article-title":"Remission of C3 glomerulopathy with rituximab as only immunosuppressive therapy.","volume":"83","author":"Giaime","year":"2015","journal-title":"Clin Nephrol"},{"key":"B20-20230813","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":"B21-20230813","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1053\/j.ajkd.2014.09.025","article-title":"Eculizumab for treatment of rapidly progressive C3 glomerulopathy.","volume":"65","author":"Le Quintrec","year":"2015","journal-title":"Am J Kidney Dis"},{"key":"B22-20230813","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1038\/nrneph.2012.214","article-title":"Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathies.","volume":"8","author":"Zuber","year":"2012","journal-title":"Nat Rev Nephrol"},{"key":"B23-20230813","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1093\/ckj\/sfv044","article-title":"Eculizumab-induced reversal of dialysis-dependent kidney failure from C3 glomerulonephritis.","volume":"8","author":"Inman","year":"2015","journal-title":"Clin Kidney J"},{"key":"B24-20230813","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":"B25-20230813","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1056\/NEJMoa1214479","article-title":"Mutations in DSTYK and dominant urinary tract malformations.","volume":"369","author":"Sanna-Cherchi","year":"2013","journal-title":"N Engl J Med"},{"key":"B26-20230813","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1038\/gim.2015.30","article-title":"Standards and guidelines for the interpretation of sequence variants: A joint consensus recommendation of the American college of medical genetics and genomics and the association for molecular pathology.","volume":"17","author":"Richards","year":"2015","journal-title":"Genet Med"},{"key":"B27-20230813","doi-asserted-by":"crossref","first-page":"461","DOI":"10.7326\/0003-4819-130-6-199903160-00002","article-title":"A more accurate method to estimate glomerular filtration rate from serum creatinine: A new prediction equation.","volume":"130","author":"Levey","year":"1999","journal-title":"Ann Intern Med"},{"key":"B28-20230813","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":"B29-20230813","doi-asserted-by":"crossref","first-page":"1103","DOI":"10.1681\/ASN.2008101028","article-title":"Mycophenolate mofetil versus cyclophosphamide for induction treatment of lupus nephritis.","volume":"20","author":"Appel","year":"2009","journal-title":"J Am Soc Nephrol"},{"key":"B30-20230813","doi-asserted-by":"crossref","first-page":"968","DOI":"10.1053\/j.ajkd.2015.02.326","article-title":"Soluble c5b-9 as a biomarker for complement activation in atypical hemolytic uremic syndrome.","volume":"65","author":"Bu","year":"2015","journal-title":"Am J Kidney Dis"},{"key":"B31-20230813","doi-asserted-by":"crossref","first-page":"3733","DOI":"10.1182\/blood-2013-12-547067","article-title":"Biomarkers of terminal complement activation confirm the diagnosis of aHUS and differentiate aHUS from TTP.","volume":"123","author":"Cataland","year":"2014","journal-title":"Blood"},{"key":"B32-20230813","doi-asserted-by":"crossref","first-page":"1245","DOI":"10.1681\/ASN.2015040385","article-title":"High-throughput genetic testing for thrombotic microangiopathies and C3 glomerulopathies.","volume":"27","author":"Bu","year":"2016","journal-title":"J Am Soc Nephrol"},{"key":"B33-20230813","article-title":"C3 glomerulonephritis and dense deposit disease share a similar disease course in a large United States cohort of C3 glomerulopathy","author":"Bomback","journal-title":"Kidney Int"}],"container-title":["Clinical Journal of the American Society of Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/01277230-201803000-00009","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T13:42:06Z","timestamp":1786369326000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/01277230-201803000-00009"}},"subtitle":["A Case Series"],"short-title":[],"issued":{"date-parts":[[2018,1,11]]},"references-count":33,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2018]]}},"URL":"https:\/\/doi.org\/10.2215\/cjn.09080817","relation":{},"ISSN":["1555-9041","1555-905X"],"issn-type":[{"value":"1555-9041","type":"print"},{"value":"1555-905X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,1,11]]}}}