{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,10]],"date-time":"2026-11-10T17:09:56Z","timestamp":1794330596411,"version":"4.9.0"},"reference-count":17,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,3,10]],"date-time":"2021-03-10T00:00:00Z","timestamp":1615334400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2021,3,10]],"date-time":"2021-03-10T00:00:00Z","timestamp":1615334400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Nephrol"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Thrombotic microangiopathy (TMA), a rare but serious complication of systemic lupus erythematosus (SLE), is associated with poor outcomes to conventional immunosuppressive therapy. Recently, eculizumab, a humanised monoclonal antibody that blocks the complement factor 5, has been known to effectively treat atypical haemolytic uremic syndrome (aHUS). Here, we report a case of aHUS co-existing with lupus nephritis that was successfully treated with eculizumab.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>A 23-year-old man presented with abdominal pain and diarrhoea. Initial laboratory tests have shown thrombocytopaenia, microangiopathic haemolytic anaemia, and acute kidney injury. Immunologic tests were consistent with SLE. Kidney biopsy have revealed lupus nephritis class IV-G with TMA. Genetic analysis have shown complement C3 gene mutations, which hints the co-existence of lupus nephritis with aHUS, a form of complement-mediated TMA. Although initial treatment with haemodialysis, plasma exchange, and conventional immunosuppressive therapy (steroid and cyclophosphamide) did not appreciably improve kidney function and thrombocytopaenia, the patient was able to respond to eculizumab therapy.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Due to the similar features of TMA and SLE, clinical suspicion of aHUS in patients with lupus nephritis is important for early diagnosis and prompt management. Timely administration of eculizumab should be considered as a treatment option for aHUS in lupus nephritis patients to yield optimal therapeutic outcomes.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12882-021-02293-2","type":"journal-article","created":{"date-parts":[[2021,3,10]],"date-time":"2021-03-10T12:03:23Z","timestamp":1615377803000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Eculizumab therapy on a patient with co\u2010existent lupus nephritis and C3 mutation\u2010related atypical haemolytic uremic syndrome: a case report"],"prefix":"10.1186","volume":"22","author":[{"given":"Mi Jung","family":"Kim","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haekyung","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yon Hee","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"So Young","family":"Jin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hee-Jin","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doyeun","family":"Oh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jin Seok","family":"Jeon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,3,10]]},"reference":[{"issue":"7","key":"2293_CR1","doi-asserted-by":"publisher","first-page":"654","DOI":"10.1056\/NEJMra1312353","volume":"371","author":"JN George","year":"2014","unstructured":"George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371(7):654\u201366.","journal-title":"N Engl J Med"},{"issue":"3","key":"2293_CR2","doi-asserted-by":"publisher","first-page":"539","DOI":"10.1016\/j.kint.2016.10.005","volume":"91","author":"TH Goodship","year":"2017","unstructured":"Goodship TH, Cook HT, Fakhouri F, Fervenza FC, Fremeaux Bacchi V, Kavanagh D, Nester CM, Noris M, Pickering MC, de Rodriguez Cordoba S, et al. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a \u201cKidney Disease: Improving Global Outcomes\u201d (KDIGO) Controversies Conference. Kidney Int. 2017;91(3):539\u201351.","journal-title":"Kidney Int."},{"issue":"4","key":"2293_CR3","doi-asserted-by":"publisher","first-page":"768","DOI":"10.1093\/rheumatology\/keq311","volume":"50","author":"MH Chen","year":"2011","unstructured":"Chen MH, Chen MH, Chen WS, Mu-Hsin Chang P, Lee HT, Lin HY, Huang DF. Thrombotic microangiopathy in systemic lupus erythematosus: a cohort study in North Taiwan. Rheumatology. 2011;50(4):768\u201375.","journal-title":"Rheumatology"},{"issue":"23","key":"2293_CR4","doi-asserted-by":"publisher","first-page":"2169","DOI":"10.1056\/NEJMoa1208981","volume":"368","author":"CM Legendre","year":"2013","unstructured":"Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, Bingham C, Cohen DJ, Delmas Y, Douglas K, et al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 2013;368(23):2169\u201381.","journal-title":"N Engl J Med"},{"issue":"4","key":"2293_CR5","doi-asserted-by":"publisher","first-page":"826","DOI":"10.1016\/j.kint.2019.07.006","volume":"96","author":"F Caravaca-Fontan","year":"2019","unstructured":"Caravaca-Fontan F, Praga M. Complement inhibitors are useful in secondary hemolytic uremic syndromes. Kidney Int. 2019;96(4):826\u20139.","journal-title":"Kidney Int"},{"issue":"4","key":"2293_CR6","doi-asserted-by":"publisher","first-page":"829","DOI":"10.1016\/j.kint.2019.08.001","volume":"96","author":"C Duineveld","year":"2019","unstructured":"Duineveld C, Wetzels JFM. Complement inhibitors are not useful in secondary hemolytic uremic syndromes. Kidney Int. 2019;96(4):829\u201333.","journal-title":"Kidney Int"},{"issue":"1","key":"2293_CR7","doi-asserted-by":"publisher","first-page":"74","DOI":"10.1016\/j.semarthrit.2018.11.005","volume":"49","author":"N Kello","year":"2019","unstructured":"Kello N, Khoury LE, Marder G, Furie R, Zapantis E, Horowitz DL. Secondary thrombotic microangiopathy in systemic lupus erythematosus and antiphospholipid syndrome, the role of complement and use of eculizumab: Case series and review of literature. Semin Arthritis Rheum. 2019;49(1):74\u201383.","journal-title":"Semin Arthritis Rheum"},{"issue":"8","key":"2293_CR8","doi-asserted-by":"publisher","first-page":"743","DOI":"10.1097\/JCMA.0000000000000344","volume":"83","author":"WC Chen","year":"2020","unstructured":"Chen WC, Ko PS, Wang HY, Liu YC, Hsiao LT, Gau JP, Liu JH. Difference in thrombotic microangiopathy between concurrently and previously diagnosed systemic lupus erythematosus. J Chin Med Assoc. 2020;83(8):743\u201350.","journal-title":"J Chin Med Assoc"},{"issue":"6","key":"2293_CR9","doi-asserted-by":"publisher","first-page":"507","DOI":"10.1111\/nep.13234","volume":"23","author":"LC Fox","year":"2018","unstructured":"Fox LC, Cohney SJ, Kausman JY, Shortt J, Hughes PD, Wood EM, Isbel NM, de Malmanche T, Durkan A, Hissaria P, et al. Consensus opinion on diagnosis and management of thrombotic microangiopathy in Australia and New Zealand. Nephrology (Carlton). 2018;23(6):507\u201317.","journal-title":"Nephrology (Carlton)"},{"issue":"6","key":"2293_CR10","doi-asserted-by":"publisher","first-page":"1443","DOI":"10.1016\/j.kint.2019.01.023","volume":"95","author":"A Le Clech","year":"2019","unstructured":"Le Clech A, Simon-Tillaux N, Provot F, Delmas Y, Vieira-Martins P, Limou S, Halimi JM, Le Quintrec M, Lebourg L, Grange S, et al. Atypical and secondary hemolytic uremic syndromes have a distinct presentation and no common genetic risk factors. Kidney Int. 2019;95(6):1443\u201352.","journal-title":"Kidney Int"},{"issue":"16","key":"2293_CR11","doi-asserted-by":"publisher","first-page":"2090","DOI":"10.1182\/bloodadvances.2018019596","volume":"2","author":"MH Park","year":"2018","unstructured":"Park MH, Caselman N, Ulmer S, Weitz IC. Complement-mediated thrombotic microangiopathy associated with lupus nephritis. Blood Adv. 2018;2(16):2090\u20134.","journal-title":"Blood Adv"},{"issue":"13","key":"2293_CR12","doi-asserted-by":"publisher","first-page":"4948","DOI":"10.1182\/blood-2008-01-133702","volume":"112","author":"V Fremeaux-Bacchi","year":"2008","unstructured":"Fremeaux-Bacchi V, Miller EC, Liszewski MK, Strain L, Blouin J, Brown AL, Moghal N, Kaplan BS, Weiss RA, Lhotta K, et al. Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome. Blood. 2008;112(13):4948\u201352.","journal-title":"Blood"},{"issue":"3","key":"2293_CR13","doi-asserted-by":"publisher","first-page":"218","DOI":"10.5045\/br.2019.54.3.218","volume":"54","author":"J Oh","year":"2019","unstructured":"Oh J, Oh D, Lee SJ, Kim JO, Kim NK, Chong SY, Huh JY, Baker RI, Korean TTPRI. Prognostic utility of ADAMTS13 activity for the atypical hemolytic uremic syndrome (aHUS) and comparison of complement serology between aHUS and thrombotic thrombocytopenic purpura. Blood Res. 2019;54(3):218\u201328.","journal-title":"Blood Res"},{"issue":"2","key":"2293_CR14","doi-asserted-by":"publisher","first-page":"238","DOI":"10.1016\/j.molimm.2012.12.006","volume":"54","author":"X Fan","year":"2013","unstructured":"Fan X, Yoshida Y, Honda S, Matsumoto M, Sawada Y, Hattori M, Hisanaga S, Hiwa R, Nakamura F, Tomomori M, et al. Analysis of genetic and predisposing factors in Japanese patients with atypical hemolytic uremic syndrome. Mol Immunol. 2013;54(2):238\u201346.","journal-title":"Mol Immunol"},{"issue":"11","key":"2293_CR15","doi-asserted-by":"publisher","first-page":"2983","DOI":"10.1111\/trf.12708","volume":"54","author":"S Rawal","year":"2014","unstructured":"Rawal S, Einbinder Y, Rubin L, Perl J, Trinkaus M, Teitel J, Pavenski K. Thrombotic microangiopathy in a patient with adult-onset Still\u2019s disease. Transfusion. 2014;54(11):2983\u20137.","journal-title":"Transfusion"},{"issue":"1","key":"2293_CR16","doi-asserted-by":"publisher","first-page":"127","DOI":"10.1053\/j.ajkd.2014.07.031","volume":"65","author":"A El-Husseini","year":"2015","unstructured":"El-Husseini A, Hannan S, Awad A, Jennings S, Cornea V, Sawaya BP. Thrombotic microangiopathy in systemic lupus erythematosus: efficacy of eculizumab. Am J Kidney Dis. 2015;65(1):127\u201330.","journal-title":"Am J Kidney Dis"},{"issue":"1","key":"2293_CR17","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1186\/s12882-020-01888-5","volume":"21","author":"RD Wright","year":"2020","unstructured":"Wright RD, Bannerman F, Beresford MW, Oni L. A systematic review of the role of eculizumab in systemic lupus erythematosus-associated thrombotic microangiopathy. BMC Nephrol. 2020;21(1):245.","journal-title":"BMC Nephrol"}],"container-title":["BMC Nephrology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s12882-021-02293-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s12882-021-02293-2\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s12882-021-02293-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,3,10]],"date-time":"2021-03-10T12:13:40Z","timestamp":1615378420000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcnephrol.biomedcentral.com\/articles\/10.1186\/s12882-021-02293-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,3,10]]},"references-count":17,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["2293"],"URL":"https:\/\/doi.org\/10.1186\/s12882-021-02293-2","relation":{},"ISSN":["1471-2369"],"issn-type":[{"value":"1471-2369","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,3,10]]},"assertion":[{"value":"25 November 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"3 March 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"10 March 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This case report was approved by the Institutional Review Board of Soonchunhyang University Hospital.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient for publication of the case report and any accompanying images. A copy of the consent form is available for review and can be provided on request.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"86"}}