{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T17:17:36Z","timestamp":1781630256226,"version":"3.54.5"},"reference-count":32,"publisher":"BMJ","issue":"11","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2024,10,22]]},"published-print":{"date-parts":[[2024,11]]},"abstract":"<jats:p>Adult-onset Still\u2019s disease (AOSD) is a rare, systemic inflammatory disorder characterised by daily fever, arthritis, a salmon-pink rash and leucocytosis. Thrombotic thrombocytopenic purpura (TTP) is included in the class of thrombotic microangiopathies and manifests clinically as microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and ischaemic tissue injury secondary to microthrombi. TTP is caused by either an autoimmune, congenital or idiopathic deficiency of ADAMTS13 and carries a high mortality rate. TTP can be seen in patients diagnosed with other rheumatologic conditions, with systemic lupus erythematosus being the most common, but is rarely seen in AOSD. Permanent complication rates from TTP in the setting of AOSD have been reported to be &gt;50%. This case study aims to report one of the rare instances of TTP presenting in a patient with known AOSD to broaden the knowledge base regarding these rare coexisting pathologies.<\/jats:p>","DOI":"10.1136\/bcr-2024-260691","type":"journal-article","created":{"date-parts":[[2024,11,9]],"date-time":"2024-11-09T01:30:19Z","timestamp":1731115819000},"page":"e260691","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":2,"title":["Thrombotic thrombocytopenic purpura in a patient with adult-onset Still\u2019s disease: report of a rare entity"],"prefix":"10.1136","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-0686-7461","authenticated-orcid":false,"given":"Kirsten","family":"Maddox","sequence":"first","affiliation":[{"name":"Louisiana State University Health Sciences Center Shreveport, Shreveport, Louisiana, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0565-8752","authenticated-orcid":false,"given":"Anil","family":"Ananthaneni","sequence":"additional","affiliation":[{"name":"Hematology\/Oncology, Louisiana State University Health Sciences Center Shreveport, Shreveport, Louisiana, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-3004-8919","authenticated-orcid":false,"given":"Neelay","family":"Patel","sequence":"additional","affiliation":[{"name":"Hematology\/Oncology, Louisiana State University Health Sciences Center Shreveport, Shreveport, Louisiana, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7547-2315","authenticated-orcid":false,"given":"Poornima","family":"Ramadas","sequence":"additional","affiliation":[{"name":"Hematology\/Oncology, Louisiana State University Health Sciences Center Shreveport, Shreveport, Louisiana, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2024,11,8]]},"reference":[{"key":"2025082623370918000_17.11.e260691.1","first-page":"424","article-title":"Preliminary criteria for classification of adult Still\u2019s disease","volume":"19","author":"Yamaguchi","year":"1992","journal-title":"J Rheumatol"},{"key":"2025082623370918000_17.11.e260691.2","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-200205000-00003"},{"key":"2025082623370918000_17.11.e260691.3","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2014.01.058"},{"key":"2025082623370918000_17.11.e260691.4","doi-asserted-by":"crossref","first-page":"583","DOI":"10.1007\/s00508-009-1217-4","article-title":"Thrombotic microangiopathy in adult-onset Still\u2019s disease: case report and review of the literature","volume":"121","author":"Salamon","year":"2009","journal-title":"Wien Klin Wochenschr"},{"key":"2025082623370918000_17.11.e260691.5","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1016\/j.semarthrit.2019.04.004","article-title":"Racial\/Ethnic variations in morbidity and mortality in Adult Onset Still\u2019s Disease: An analysis of national dataset","volume":"49","author":"Mehta","year":"2019","journal-title":"Semin Arthritis Rheum"},{"key":"2025082623370918000_17.11.e260691.6","article-title":"Adult Onset Still\u2019s Disease: A Review on Diagnostic Workup and Treatment Options","volume":"2016","author":"Gopalarathinam","year":"2016","journal-title":"Case Rep Rheumatol"},{"key":"2025082623370918000_17.11.e260691.7","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1312353"},{"key":"2025082623370918000_17.11.e260691.8","doi-asserted-by":"publisher","DOI":"10.1097\/MNH.0b013e3283378f25"},{"key":"2025082623370918000_17.11.e260691.9","doi-asserted-by":"crossref","first-page":"e237","DOI":"10.1016\/S2352-3026(16)30018-7","article-title":"Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy","volume":"3","author":"Mariotte","year":"2016","journal-title":"Lancet Haematol"},{"key":"2025082623370918000_17.11.e260691.10","unstructured":"Nu\u00f1ez Zuno JA , Khaddour K . 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