{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T02:46:05Z","timestamp":1780368365486,"version":"3.54.1"},"reference-count":28,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2021,4,20]],"date-time":"2021-04-20T00:00:00Z","timestamp":1618876800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2021,4,20]],"date-time":"2021-04-20T00:00:00Z","timestamp":1618876800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Eye"],"published-print":{"date-parts":[[2022,4]]},"DOI":"10.1038\/s41433-021-01464-7","type":"journal-article","created":{"date-parts":[[2021,4,20]],"date-time":"2021-04-20T17:56:11Z","timestamp":1618941371000},"page":"781-788","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Susac\u2019s syndrome \u2013 A new ocular finding and disease outcome"],"prefix":"10.1038","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1147-444X","authenticated-orcid":false,"family":"Dinah Zur","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"family":"Michaella Goldstein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8879-5378","authenticated-orcid":false,"given":"Dana","family":"Barequet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yahav","family":"Oron","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ori","family":"Elkayam","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnon","family":"Karni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adi","family":"Wilf-Yarkoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keren","family":"Regev","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zohar","family":"Habot-Wilner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,4,20]]},"reference":[{"key":"1464_CR1","doi-asserted-by":"publisher","first-page":"591","DOI":"10.1212\/WNL.44.4.591","volume":"44","author":"JO Susac","year":"1994","unstructured":"Susac JO. Susac\u2019s syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology. 1994;44:591\u20133. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/8164809","journal-title":"Neurology."},{"key":"1464_CR2","doi-asserted-by":"publisher","first-page":"60","DOI":"10.1097\/WNO.0000000000000677","volume":"39","author":"RA Egan","year":"2019","unstructured":"Egan RA. Diagnostic criteria and treatment algorithm for Susac syndrome. J Neuroophthalmol. 2019;39:60\u201367. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/29933288","journal-title":"J Neuroophthalmol."},{"key":"1464_CR3","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-019-13593-5","volume":"10","author":"CC Gross","year":"2019","unstructured":"Gross CC, Meyer C, Bhatia U, Yshii L, Kleffner I, Bauer J, et al. CD8+ T cell-mediated endotheliopathy is a targetable mechanism of neuro-inflammation in Susac syndrome. Nat Commun. 2019;10:5779. https:\/\/doi.org\/10.1038\/s41467-019-13593-5","journal-title":"Nat Commun"},{"key":"1464_CR4","doi-asserted-by":"publisher","DOI":"10.1186\/1742-2094-11-46","volume":"11","author":"S Jarius","year":"2014","unstructured":"Jarius S, Kleffner I, D\u00f6rr JM, Sastre-Garriga J, Illes Z, Eggenberger E, et al. Clinical, paraclinical and serological findings in Susac syndrome: an international multicenter study. J Neuroinflammation. 2014;11:46. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/24606999","journal-title":"J Neuroinflammation"},{"key":"1464_CR5","doi-asserted-by":"publisher","first-page":"259","DOI":"10.1016\/j.jns.2009.07.002","volume":"285","author":"S Jarius","year":"2009","unstructured":"Jarius S, Neumayer B, Wandinger KP, Hartmann M, Wildemann B. Anti-endothelial serum antibodies in a patient with Susac\u2019s syndrome. J Neurol Sci. 2009;285:259\u201361. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/19643446","journal-title":"J Neurol Sci."},{"key":"1464_CR6","doi-asserted-by":"publisher","first-page":"903","DOI":"10.1309\/AJCPERI7LC4VNFYK","volume":"136","author":"CM Magro","year":"2011","unstructured":"Magro CM, Poe JC, Lubow M, Susac JO. Susac syndrome: an organ-specific autoimmune endotheliopathy syndrome associated with anti-endothelial cell antibodies. Am J Clin Pathol. 2011;136:903\u201312. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/22095376","journal-title":"Am J Clin Pathol"},{"key":"1464_CR7","doi-asserted-by":"publisher","first-page":"1287","DOI":"10.1136\/jnnp-2016-314295","volume":"87","author":"I Kleffner","year":"2016","unstructured":"Kleffner I, D\u00f6rr J, Ringelstein M, Gross CC, B\u00f6ckenfeld Y, Schwindt W, et al. Diagnostic criteria for Susac syndrome. J Neurol Neurosurg Psychiatry. 2016;87:1287\u201395. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/28103199","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"1464_CR8","doi-asserted-by":"publisher","first-page":"110","DOI":"10.1016\/j.msard.2013.05.005","volume":"3","author":"JT Bernard","year":"2014","unstructured":"Bernard JT, Romero R, Agrawal K, Jager R, Rezania K. Optical coherence tomography in Susac\u2019s syndrome. Mult Scler Relat Disord. 2014;3:110\u20136. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/25877981","journal-title":"Mult Scler Relat Disord."},{"key":"1464_CR9","doi-asserted-by":"publisher","first-page":"366","DOI":"10.1097\/IAE.0000000000000700","volume":"36","author":"AU Brandt","year":"2016","unstructured":"Brandt AU, Oberwahrenbrock T, Costello F, Fielden M, Gertz K, Kleffner I, et al. Retinal lesion evolution in SUSAC syndrome. Retina. 2016;36:366\u201374. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/26200513","journal-title":"Retina."},{"key":"1464_CR10","doi-asserted-by":"publisher","first-page":"67","DOI":"10.1007\/s11940-008-0008-y","volume":"10","author":"RM Rennebohm","year":"2008","unstructured":"Rennebohm RM, Egan RA, Susac JO. Treatment of Susac\u2019s syndrome. Curr Treat Options Neurol 2008;10:67\u201374. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/18325301","journal-title":"Curr Treat Options Neurol"},{"key":"1464_CR11","doi-asserted-by":"publisher","first-page":"610","DOI":"10.1212\/WNL.0000000000001852","volume":"85","author":"M Ringelstein","year":"2015","unstructured":"Ringelstein M, Albrecht P, Kleffner I, B\u00fchn B, Harmel J, M\u00fcller A-K, et al. Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography. Neurology. 2015;85:610\u20138. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/26203089","journal-title":"Neurology."},{"key":"1464_CR12","doi-asserted-by":"publisher","first-page":"234","DOI":"10.1097\/ICB.0000000000000470","volume":"12","author":"P Salvanos","year":"2018","unstructured":"Salvanos P, Moe MC, Utheim TP, Bragad\u00f3ttir R, Kerty E. Ultra-wide-field fundus imaging in the diagnosis and follow-up of Susac syndrome. Retin Cases Brief Rep. 2018;12:234\u20139. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/27828899","journal-title":"Retin Cases Brief Rep."},{"key":"1464_CR13","doi-asserted-by":"publisher","first-page":"373","DOI":"10.1046\/j.1442-9071.2000.00345.x","volume":"28","author":"VP Saw","year":"2000","unstructured":"Saw VP, Canty PA, Green CM, Briggs RJ, Cremer PD, Harrisberg B, et al. Susac syndrome: microangiopathy of the retina, cochlea and brain. Clin Exp Ophthalmol. 2000;28:373\u201381. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/11097286","journal-title":"Clin Exp Ophthalmol"},{"key":"1464_CR14","doi-asserted-by":"publisher","first-page":"505","DOI":"10.3928\/23258160-20170601-10","volume":"48","author":"K Spiess","year":"2017","unstructured":"Spiess K, Mart\u00ednez JRG. OCT angiography: assessment of retinal ischemia in Susac\u2019s syndrome. Ophthalmic Surg Lasers Imaging Retina. 2017;48:505\u20138. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/28613358","journal-title":"Ophthalmic Surg Lasers Imaging Retina."},{"key":"1464_CR15","doi-asserted-by":"publisher","first-page":"e5223","DOI":"10.1097\/MD.0000000000005223","volume":"95","author":"V Vishnevskia-Dai","year":"2016","unstructured":"Vishnevskia-Dai V, Chapman J, Sheinfeld R, Sharon T, Huna-Baron R, Manor RS, et al. Susac syndrome: clinical characteristics, clinical classification, and long-term prognosis. Medicine. 2016;95:e5223\u2013e5223. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/27787385","journal-title":"Medicine"},{"key":"1464_CR16","doi-asserted-by":"publisher","first-page":"236","DOI":"10.1097\/ICB.0000000000000344","volume":"11","author":"JB Yepez","year":"2017","unstructured":"Yepez JB, Murati FA, Pettito M, Arevalo JF. Intravitreal triamcinolone in susac syndrome. Retin Cases Brief Rep. 2017;11:236\u20139. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/27276498","journal-title":"Retin Cases Brief Rep."},{"key":"1464_CR17","doi-asserted-by":"publisher","first-page":"683","DOI":"10.1038\/nrneurol.2009.176","volume":"5","author":"J D\u00f6rr","year":"2009","unstructured":"D\u00f6rr J, Radbruch H, Bock M, Wuerfel J, Br\u00fcggemann A, Wandinger KP, et al. Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome. Nat Rev Neurol. 2009;5:683\u20138. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/19953118","journal-title":"Nat Rev Neurol"},{"key":"1464_CR18","doi-asserted-by":"publisher","first-page":"307","DOI":"10.1038\/nrneurol.2013.82","volume":"9","author":"J D\u00f6rr","year":"2013","unstructured":"D\u00f6rr J, Krautwald S, Wildemann B, Jarius S, Ringelstein M, Duning T, et al. Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 2013;9:307\u201316. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/23628737","journal-title":"Nat Rev Neurol"},{"key":"1464_CR19","doi-asserted-by":"publisher","unstructured":"Buelens T, Ossewaarde-van Norel J, de Boer JH, Nubourgh I, Glibert G, Kamgang Semeu P, et al. Evaluation of tumor necrosis factor inhibitor therapy in susac syndrome. Retina. 2019: https:\/\/doi.org\/10.1097\/IAE.0000000000002466. Available at:https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/30707148","DOI":"10.1097\/IAE.0000000000002466."},{"key":"1464_CR20","doi-asserted-by":"publisher","first-page":"483","DOI":"10.1016\/S0002-9394(02)02085-8","volume":"135","author":"RA Egan","year":"2003","unstructured":"Egan RA, Ha Nguyen T, Gass JDM, Rizzo JF 3rd, Tivnan J, Susac JO. Retinal arterial wall plaques in Susac syndrome. Am J Ophthalmol. 2003;135:483\u20136. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/12654364","journal-title":"Am J Ophthalmol"},{"key":"1464_CR21","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1016\/j.jns.2010.08.043","volume":"299","author":"RA Egan","year":"2010","unstructured":"Egan RA, Hills WL, Susac JO. Gass plaques and fluorescein leakage in Susac Syndrome. J Neurol Sci. 2010;299:97\u2013100. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/20880549","journal-title":"J Neurol Sci."},{"key":"1464_CR22","doi-asserted-by":"publisher","first-page":"459","DOI":"10.1097\/WNO.0000000000000627","volume":"38","author":"RA Egan","year":"2018","unstructured":"Egan RA, Jirawuthiworavong G, Lincoff NS, Chen JJ, Francis CE, Leavitt JA. Retinal arterio-arterial collaterals in Susac syndrome. J Neuroophthalmol. 2018;38:459\u201361. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/29341996","journal-title":"J Neuroophthalmol."},{"key":"1464_CR23","doi-asserted-by":"publisher","first-page":"477","DOI":"10.1016\/j.survophthal.2019.01.007","volume":"64","author":"LZ Heng","year":"2019","unstructured":"Heng LZ, Bailey C, Lee R, Dick A, Ross A. A review and update on the ophthalmic implications of Susac syndrome. Surv Ophthalmol. 2019;64:477\u201385. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/30703404","journal-title":"Surv Ophthalmol"},{"key":"1464_CR24","doi-asserted-by":"publisher","first-page":"1238","DOI":"10.1097\/IAE.0b013e31809ff824","volume":"27","author":"N Martinet","year":"2007","unstructured":"Martinet N, Fardeau C, Adam R, Bodaghi B, Papo T, Piette J-C, et al. Fluorescein and indocyanine green angiographies in Susac syndrome. Retina 2007;27:1238\u201342. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/18046231","journal-title":"Retina"},{"key":"1464_CR25","doi-asserted-by":"publisher","first-page":"1803","DOI":"10.1007\/s10792-017-0653-9","volume":"38","author":"R Mastropasqua","year":"2018","unstructured":"Mastropasqua R, Toto L, Senatore A, D\u2019Uffizi A, Neri P, Mariotti C, et al. Optical coherence tomography angiography findings in Susac\u2019s syndrome: a case report. Int Ophthalmol. 2018;38:1803\u20138. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/28730399","journal-title":"Int Ophthalmol"},{"key":"1464_CR26","doi-asserted-by":"publisher","first-page":"800","DOI":"10.1111\/j.1468-1331.2011.03627.x","volume":"19","author":"FJ Mateen","year":"2012","unstructured":"Mateen FJ, Zubkov AY, Muralidharan R, Fugate JE, Rodriguez FJ, Winters JL, et al. Susac syndrome: clinical characteristics and treatment in 29 new cases. Eur J Neurol. 2012;19:800\u201311. https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/22221557","journal-title":"Eur J Neurol"},{"key":"1464_CR27","unstructured":"Rennebohm RM, Asdaghi N, Srivastava S, Gertner E Guidelines for treatment of Susac syndrome - An update. Int. J. Stroke. 2018: 1747493017751737\u20131747493017751737. Available at:https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/29319463."},{"key":"1464_CR28","doi-asserted-by":"publisher","first-page":"218","DOI":"10.1159\/000493489","volume":"61","author":"T Tomiyasu","year":"2019","unstructured":"Tomiyasu T, Hirano Y, Suzuki N, Esaki Y, Yasuda Y, Uemura A, et al. Structural and functional analyses of retinal ischemia in eyes with retinal vein occlusion: relationship with macular edema or microaneurysm formation. Ophthalmic Res. 2019;61:218\u201325. https:\/\/www.karger.com\/DOI\/10.1159\/000493489","journal-title":"Ophthalmic Res."}],"container-title":["Eye"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.nature.com\/articles\/s41433-021-01464-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41433-021-01464-7","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41433-021-01464-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,3,25]],"date-time":"2022-03-25T08:04:51Z","timestamp":1648195491000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/s41433-021-01464-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,4,20]]},"references-count":28,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2022,4]]}},"alternative-id":["1464"],"URL":"https:\/\/doi.org\/10.1038\/s41433-021-01464-7","relation":{},"ISSN":["0950-222X","1476-5454"],"issn-type":[{"value":"0950-222X","type":"print"},{"value":"1476-5454","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,4,20]]},"assertion":[{"value":"19 September 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 January 2021","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"9 February 2021","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 April 2021","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with ethical standards"}},{"value":"The authors declare no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}