{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T11:44:55Z","timestamp":1791546295909,"version":"4.3.4"},"reference-count":18,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,12,28]],"date-time":"2024-12-28T00:00:00Z","timestamp":1735344000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,12,28]],"date-time":"2024-12-28T00:00:00Z","timestamp":1735344000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Med Case Reports"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Evans syndrome is a rare disorder characterized by the simultaneous or sequential combination of autoimmune hemolytic anemia and immunological thrombocytopenia, together with a positive direct antiglobulin test. This syndrome, which can be primary or secondary, is a rare initial manifestation of autoimmune diseases, notably systemic lupus erythematosus, with 1.7\u20132.7% of patients with systemic lupus erythematosus developing secondary Evans syndrome, sometimes preceding the onset of systemic lupus erythematosus symptoms.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>A 47-year-old Middle Eastern female presented with symptoms including shortness of breath, chest pain, and weakness. Physical examination revealed pallor, pale conjunctiva, icteric sclera, tachycardia, and tachypnea. She was diagnosed with Evans syndrome owing to hemolytic anemia, thrombocytopenia, and a positive Coombs test, where initial resistance to treatment prompted intensive therapy with methylprednisolone, intravenous immunoglobulin, and rituximab. Subsequent identification of systemic lupus erythematosus on the basis of positive anti-nuclear antibodies and anti-double-stranded deoxyribonucleic acid antibodies led to treatment with mycophenolate mofetil and hydroxychloroquine. Further evaluations uncovered Hashimoto\u2019s thyroiditis and pernicious anemia, necessitating thyroxine supplementation and vitamin B12 injections.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>Hematologic abnormalities play a crucial role in the diagnostic framework of systemic lupus erythematosus. This case highlights a patient initially diagnosed with Evans syndrome, revealing an underlying systemic lupus erythematosus. The presentation underscores the significance of hematologic manifestations as integral components of the diagnostic framework for autoimmune diseases, emphasizing the intricate relationship between Evans syndrome and systemic lupus erythematosus.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13256-024-05002-3","type":"journal-article","created":{"date-parts":[[2024,12,28]],"date-time":"2024-12-28T16:15:46Z","timestamp":1735402546000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Evans syndrome as a presentation in systemic lupus erythematous, coexisting with Hashimoto\u2019s thyroiditis and pernicious anemia: a case report"],"prefix":"10.1186","volume":"18","author":[{"given":"Maryam","family":"Mansour","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arwa","family":"Shamasnah","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deema","family":"Alsaadi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saja","family":"Abu Saif","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akram","family":"krama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,12,28]]},"reference":[{"issue":"1","key":"5002_CR1","doi-asserted-by":"publisher","first-page":"48","DOI":"10.1001\/archinte.1951.03810010058005","volume":"87","author":"RS Evans","year":"1951","unstructured":"Evans RS, Takahashi K, Duane RT, Payne R, Liu C. Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology. AMA Arch Intern Med. 1951;87(1):48\u201365.","journal-title":"AMA Arch Intern Med"},{"issue":"10","key":"5002_CR2","doi-asserted-by":"publisher","first-page":"1081","DOI":"10.1002\/ajh.25574","volume":"94","author":"DL Hansen","year":"2019","unstructured":"Hansen DL, M\u00f6ller S, Andersen K, Gaist D, Frederiksen H. Evans syndrome in adults\u2014incidence, prevalence, and survival in a nationwide cohort. Am J Hematol. 2019;94(10):1081\u201390.","journal-title":"Am J Hematol"},{"key":"5002_CR3","doi-asserted-by":"publisher","DOI":"10.3390\/jcm9123851","author":"S Audia","year":"2020","unstructured":"Audia S, Grienay N, Mounier M, Michel M, Bonnotte B. Evans\u2019 Syndrome: from diagnosis to treatment. J Clin Med. 2020. https:\/\/doi.org\/10.3390\/jcm9123851.","journal-title":"J Clin Med"},{"issue":"1","key":"5002_CR4","doi-asserted-by":"publisher","first-page":"147","DOI":"10.4103\/1319-2442.174177","volume":"27","author":"S Mendonca","year":"2016","unstructured":"Mendonca S, Srivastava S, Kapoor R, Gupta D, Gupta P, Sharma ML. Evans syndrome and its link with systemic lupus erythematosus. Saudi J Kidney Dis Transpl. 2016;27(1):147\u20139.","journal-title":"Saudi J Kidney Dis Transpl"},{"issue":"15","key":"5002_CR5","doi-asserted-by":"publisher","first-page":"3167","DOI":"10.1182\/blood-2009-04-215368","volume":"114","author":"M Michel","year":"2009","unstructured":"Michel M, Chanet V, Dechartres A, Morin AS, Piette JC, Cirasino L, Emilia G, Zaja F, Ruggeri M, Andr\u00e8s E, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood. 2009;114(15):3167\u201372.","journal-title":"Blood"},{"issue":"4","key":"5002_CR6","doi-asserted-by":"publisher","first-page":"230","DOI":"10.1016\/j.bjhh.2015.03.002","volume":"37","author":"JC Jaime-P\u00e9rez","year":"2015","unstructured":"Jaime-P\u00e9rez JC, Guerra-Leal LN, L\u00f3pez-Razo ON, M\u00e9ndez-Ram\u00edrez N, G\u00f3mez-Almaguer D. Experience with Evans syndrome in an academic referral center. Rev Bras Hematol Hemoter. 2015;37(4):230\u20135.","journal-title":"Rev Bras Hematol Hemoter"},{"issue":"1","key":"5002_CR7","doi-asserted-by":"publisher","first-page":"68","DOI":"10.1007\/s12288-014-0357-5","volume":"31","author":"N Thakur","year":"2015","unstructured":"Thakur N, Chandra J, Dhingra B, Singh V. Pediatric lupus: varied haematological picture and presentation. Indian J Hematol Blood Transfusion. 2015;31(1):68\u201370.","journal-title":"Indian J Hematol Blood Transfusion"},{"issue":"4","key":"5002_CR8","doi-asserted-by":"publisher","first-page":"362","DOI":"10.1016\/j.jbspin.2011.07.004","volume":"79","author":"GL Costallat","year":"2012","unstructured":"Costallat GL, Appenzeller S, Costallat LT. Evans syndrome and systemic lupus erythematosus: clinical presentation and outcome. Joint Bone Spine. 2012;79(4):362\u20134.","journal-title":"Joint Bone Spine"},{"issue":"7","key":"5002_CR9","doi-asserted-by":"publisher","first-page":"1238","DOI":"10.1002\/pbc.25976","volume":"63","author":"GE Lube","year":"2016","unstructured":"Lube GE, Ferriani MP, Campos LM, Terreri MT, Bonf\u00e1 E, Magalh\u00e3es CS, Aikawa NE, Piotto DP, Peracchi OA, Dos Santos MC, et al. Evans syndrome at childhood-onset systemic lupus erythematosus diagnosis: a large multicenter study. Pediatr Blood Cancer. 2016;63(7):1238\u201343.","journal-title":"Pediatr Blood Cancer"},{"issue":"5","key":"5002_CR10","doi-asserted-by":"publisher","first-page":"862","DOI":"10.1016\/j.jaad.2015.12.029","volume":"74","author":"J Tiao","year":"2016","unstructured":"Tiao J, Feng R, Carr K, Okawa J, Werth VP. Using the American college of Rheumatology (ACR) and systemic lupus international collaborating clinics (SLICC) criteria to determine the diagnosis of systemic lupus erythematosus (SLE) in patients with subacute cutaneous lupus erythematosus (SCLE). J Am Acad Dermatol. 2016;74(5):862\u20139.","journal-title":"J Am Acad Dermatol"},{"issue":"3","key":"5002_CR11","first-page":"189","volume":"14","author":"SC Ng","year":"1992","unstructured":"Ng SC. Evans syndrome: a report on 12 patients. Clin Lab Haematol. 1992;14(3):189\u201393.","journal-title":"Clin Lab Haematol"},{"issue":"3","key":"5002_CR12","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1136\/adc.77.3.245","volume":"77","author":"S Sava\u015fan","year":"1997","unstructured":"Sava\u015fan S, Warrier I, Ravindranath Y. The spectrum of Evans\u2019 syndrome. Arch Dis Child. 1997;77(3):245\u20138.","journal-title":"Arch Dis Child"},{"issue":"3","key":"5002_CR13","doi-asserted-by":"publisher","first-page":"445","DOI":"10.1111\/j.1365-2141.1982.00445.x","volume":"51","author":"JG Pegels","year":"1982","unstructured":"Pegels JG, Helmerhorst FM, van Leeuwen EF, van de Plas-van DC, Engelfriet CP, von dem Borne AE. The Evans syndrome: characterization of the responsible autoantibodies. Br J Haematol. 1982;51(3):445\u201350.","journal-title":"Br J Haematol"},{"issue":"4","key":"5002_CR14","doi-asserted-by":"publisher","first-page":"330","DOI":"10.1097\/00043426-198824000-00013","volume":"10","author":"WC Wang","year":"1988","unstructured":"Wang WC. Evans syndrome in childhood: pathophysiology, clinical course, and treatment. Am J Pediatr Hematol Oncol. 1988;10(4):330\u20138.","journal-title":"Am J Pediatr Hematol Oncol"},{"issue":"3","key":"5002_CR15","doi-asserted-by":"publisher","first-page":"329","DOI":"10.1182\/blood.V56.3.329.329","volume":"56","author":"S Karpatkin","year":"1980","unstructured":"Karpatkin S. Autoimmune thrombocytopenic purpura. Blood. 1980;56(3):329\u201343.","journal-title":"Blood"},{"issue":"11","key":"5002_CR16","doi-asserted-by":"publisher","first-page":"1271","DOI":"10.1002\/art.1780251101","volume":"25","author":"EM Tan","year":"1982","unstructured":"Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, Schaller JG, Talal N, Winchester RJ. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25(11):1271\u20137.","journal-title":"Arthritis Rheum"},{"issue":"2","key":"5002_CR17","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1111\/j.1365-2141.2005.05809.x","volume":"132","author":"A Norton","year":"2006","unstructured":"Norton A, Roberts I. Management of Evans syndrome. Br J Haematol. 2006;132(2):125\u201337.","journal-title":"Br J Haematol"},{"key":"5002_CR18","doi-asserted-by":"publisher","first-page":"139","DOI":"10.1016\/j.jaut.2016.07.001","volume":"74","author":"A Velo-Garc\u00eda","year":"2016","unstructured":"Velo-Garc\u00eda A, Castro SG, Isenberg DA. The diagnosis and management of the haematologic manifestations of lupus. J Autoimmun. 2016;74:139\u201360.","journal-title":"J Autoimmun"}],"container-title":["Journal of Medical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13256-024-05002-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13256-024-05002-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13256-024-05002-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,12,28]],"date-time":"2024-12-28T17:02:07Z","timestamp":1735405327000},"score":1,"resource":{"primary":{"URL":"https:\/\/jmedicalcasereports.biomedcentral.com\/articles\/10.1186\/s13256-024-05002-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,12,28]]},"references-count":18,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["5002"],"URL":"https:\/\/doi.org\/10.1186\/s13256-024-05002-3","relation":{},"ISSN":["1752-1947"],"issn-type":[{"value":"1752-1947","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,12,28]]},"assertion":[{"value":"18 December 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"25 November 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 December 2024","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 article does not contain any studies with human participants or animals performed by any of the authors.","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 this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"Authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"643"}}