{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T02:22:22Z","timestamp":1781835742033,"version":"3.54.5"},"reference-count":12,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2020,8,24]],"date-time":"2020-08-24T00:00:00Z","timestamp":1598227200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,8,24]],"date-time":"2020-08-24T00:00:00Z","timestamp":1598227200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"crossref","award":["No.81670697"],"award-info":[{"award-number":["No.81670697"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Nephrol"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n<jats:title>Background<\/jats:title>\n<jats:p>Anti-low density lipoprotein receptor-related protein 2 (LRP2) nephropathy\/anti-brush border antibody (ABBA) disease is a disorder characterized by acute tubulointerstitial injury associated with circulating antibodies to kidney proximal tubular brush border protein LRP2\/megalin. Patients are typically elderly and present with acute kidney injury and subnephrotic proteinuria. They progress to end-stage renal disease with poor response to immunosuppressive therapies.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Case presentation<\/jats:title>\n<jats:p>We report a case of a 29-year-old Chinese woman, who presented with nephrotic syndrome with normal kidney function. Kidney biopsy showed no obvious tubular injury or interstitial inflammation. Positive immunoglobulin G (IgG) staining was revealed along the brush border of proximal tubular cells. Anti-LRP2 antibody was identified in serum, consistent with a diagnosis of anti-LRP2 nephropathy. The patient achieved complete remission after receiving prednisone and cyclophosphamide.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>Anti-LRP2 nephropathy can also present as nephrotic syndrome in young patients and complete remission from nephrotic syndrome may be achieved after immunosuppressive therapy.<\/jats:p>\n<\/jats:sec>","DOI":"10.1186\/s12882-020-02027-w","type":"journal-article","created":{"date-parts":[[2020,8,24]],"date-time":"2020-08-24T13:02:36Z","timestamp":1598274156000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Complete remission of nephrotic syndrome in a young woman with anti-LRP2 nephropathy after immunosuppressive therapy"],"prefix":"10.1186","volume":"21","author":[{"given":"Xiaoye","family":"Zhu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lingxue","family":"Tu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shaojun","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huaizhou","family":"You","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun","family":"Xue","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chuanming","family":"Hao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,8,24]]},"reference":[{"key":"2027_CR1","doi-asserted-by":"publisher","first-page":"1326","DOI":"10.1056\/NEJM198111263052206","volume":"305","author":"MFS Douglas","year":"1981","unstructured":"Douglas MFS, Rabideau DP, Schwartz MM, Lewis EJ. Evidence on autologous immunecomlplex nephritis. N Engl J Med. 1981;305:1326\u20139.","journal-title":"N Engl J Med"},{"key":"2027_CR2","doi-asserted-by":"publisher","first-page":"602","DOI":"10.1093\/ajcp\/75.4.602","volume":"75","author":"EB Morrison","year":"1981","unstructured":"Morrison EB, Kozlowski EJ, McPhaul JJ Jr. Primary tubulointerstitial nephritis caused by antibodies to proximal tubular antigens. Am J Clin Pathol. 1981;75:602\u20139.","journal-title":"Am J Clin Pathol"},{"key":"2027_CR3","doi-asserted-by":"publisher","first-page":"380","DOI":"10.1681\/ASN.2015030334","volume":"27","author":"IA Rosales","year":"2016","unstructured":"Rosales IA, Collins AB, do Carmo PA, Tolkoff-Rubin N, Smith RN, Colvin RB. Immune complex tubulointerstitial nephritis due to autoantibodies to the proximal tubule brush border. J Am Soc Nephrol. 2016;27:380\u20134.","journal-title":"J Am Soc Nephrol"},{"key":"2027_CR4","doi-asserted-by":"publisher","first-page":"644","DOI":"10.1681\/ASN.2017060664","volume":"29","author":"CP Larsen","year":"2018","unstructured":"Larsen CP, Trivin-Avillach C, Coles P, et al. LDL receptor-related protein 2(Megalin) as a target antigen in human kidney anti-brush border antibody disease. J Am Soc Nephrol. 2018;29:644\u201353.","journal-title":"J Am Soc Nephrol"},{"key":"2027_CR5","doi-asserted-by":"publisher","first-page":"132","DOI":"10.1053\/j.ajkd.2018.12.039","volume":"74","author":"KP Dinesh","year":"2019","unstructured":"Dinesh KP, Raniele D, Michels K, et al. Anti-LRP2 nephropathy with abundant IgG4-positive plasma cells: a case report. Am J Kidney Dis. 2019;74:132\u20137.","journal-title":"Am J Kidney Dis"},{"key":"2027_CR6","doi-asserted-by":"crossref","unstructured":"Gamayo A, Hecox D, Andeen NK, et al. 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IgG subclass staining in renal biopsies with membrane glomerulonephritis indicates subclass switch during disease progression. Mod Pathol. 2013;26(6):799\u2013805.","journal-title":"Mod Pathol"},{"issue":"11","key":"2027_CR10","doi-asserted-by":"publisher","first-page":"1555","DOI":"10.1016\/j.ekir.2019.07.015","volume":"4","author":"CB Drachenberg","year":"2019","unstructured":"Drachenberg CB, Papadimitriou JC, Rubin MF, et al. Epidemiology and pathophsiology of glomerular C4d staining in native kidney biopsies. Kidney Int Rep. 2019;4(11):1555\u201367.","journal-title":"Kidney Int Rep"},{"issue":"9","key":"2027_CR11","doi-asserted-by":"publisher","first-page":"e25065","DOI":"10.1371\/journal.pone.0025065","volume":"6","author":"T Prabakaran","year":"2011","unstructured":"Prabakaran T, Nielsen R, Larsen JV, et al. Receptor-mediated endocytosis of \u03b1-galactosidase a in human podocytes in fabry disease. 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Written informed consent was obtained from the patient prior to her inclusion in the study.","order":1,"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.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"364"}}