{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T17:16:15Z","timestamp":1784740575829,"version":"3.55.0"},"reference-count":20,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2023,2,8]],"date-time":"2023-02-08T00:00:00Z","timestamp":1675814400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,6,19]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>An 18-year-old Japanese woman with systemic lupus erythematosus experienced dyspnoea, headache, tinnitus, and purpura for 2 weeks and was admitted to our hospital. The patient had been diagnosed with systemic lupus erythematosus and secondary immune thrombocytopenia 8 years before and treated with high-dose prednisolone and mycophenolate mofetil. Since the blood test on admission showed haemolytic anaemia with a positive direct Coombs test and anti-glycoprotein IIb\/IIIa antibodies, the patient was initially diagnosed with Evans syndrome (ES). The patient was treated with pulse intravenous methylprednisolone followed by 45\u2009mg\/day prednisolone; however, the patient\u2019s platelet count did not normalise. Based on a low level of a disintegrin-like and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS-13) activity and a high level of ADAMTS-13 inhibitors, a diagnosis of acquired thrombotic thrombocytopenic purpura (TTP) was confirmed. After undergoing therapeutic plasma exchange for 6 consecutive days, the patient\u2019s platelet count recovered rapidly. Although concurrent acquired TTP and ES have not been reported previously, the findings from this case highlight the importance of measuring ADAMTS-13 activity and inhibitors to rule out acquired TTP, especially when ES is refractory to glucocorticoids.<\/jats:p>","DOI":"10.1093\/mrcr\/rxad011","type":"journal-article","created":{"date-parts":[[2023,2,13]],"date-time":"2023-02-13T12:23:22Z","timestamp":1676291002000},"page":"383-387","source":"Crossref","is-referenced-by-count":5,"title":["A case of systemic lupus erythematosus having concurrent Evans syndrome and acquired thrombotic thrombocytopenic purpura"],"prefix":"10.1093","volume":"7","author":[{"given":"Ryo","family":"Motoyama","sequence":"first","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Tokyo Women\u2019s Medical University School of Medicine , Tokyo, 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