{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T11:40:31Z","timestamp":1790595631225,"version":"4.1.0"},"reference-count":35,"publisher":"BMJ","issue":"3","license":[{"start":{"date-parts":[[2024,8,29]],"date-time":"2024-08-29T00:00:00Z","timestamp":1724889600000},"content-version":"unspecified","delay-in-days":28,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["RMD Open"],"accepted":{"date-parts":[[2024,8,2]]},"published-print":{"date-parts":[[2024,8]]},"abstract":"<jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>The association between immune-mediated thrombotic thrombocytopenic purpura (iTTP) and Sj\u00f6gren disease (SjD) has been poorly investigated. This study presents the first retrospective cohort of iTTP-SjD aiming to identify risk factors for iTTP occurrence in SjD patients and examine their clinical course.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>Patients with iTTP-SjD were identified within the French TTP Registry based on American College of Rheumatology\/European League Against Rheumatism 2016 criteria. A comparative analysis was conducted with two control groups comprising primary SjD (pSjD) patients from the French ASSESS cohort and idiopathic iTTP patients from the French TTP Registry. Demographic, clinical and biological data were retrospectively collected.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Thirty iTTP-SjD patients were included and compared with 65 pSjD and 45 idiopathic iTTP patients. The majority of iTTP-SjD patients (n=18) were diagnosed with SjD at the time of iTTP diagnosis. In comparison with the pSjD cohort, iTTP-SjD patients were diagnosed with SjD at a younger age (p=0.039) and showed a higher prevalence of anti-SjS-related antigen A antibody positivity and xerostomia (p=0.015, p=0.035, respectively). EULAR Sjogren\u2019s Syndrome Disease Activity Index showed similar activity levels between the two groups. iTTP-SjD patients were treated with plasma exchange (n=28), corticosteroids, rituximab (n=19) and caplacizumab (n=3). In comparison with the idiopathic iTTP cohort, mortality rates (log-rank tests, p=0.228), biological and clinical iTTP relapses (multivariate analysis, p=0.181) were comparable and short-term outcomes (survival at day 30, relapse) were favourable.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>iTTP can be a rare complication in patients with SjD. Further studies involving larger cohorts and long-term follow-up are warranted to confirm these findings and to explore the efficacy of immunomodulators and caplacizumab in iTTP-SjD patients.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1136\/rmdopen-2024-004426","type":"journal-article","created":{"date-parts":[[2024,8,29]],"date-time":"2024-08-29T22:25:15Z","timestamp":1724970315000},"page":"e004426","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":3,"title":["Clinical, biological, prognostic characteristics of patients with immune-mediated thrombotic thrombocytopenic purpura and Sj\u00f6gren\u2019s disease"],"prefix":"10.1136","volume":"10","author":[{"given":"Justine","family":"Luciano","sequence":"first","affiliation":[{"name":"Inserm, CEA, Immunologie des maladies virales, auto-immunes, h\u00e9matologiques et bact\u00e9riennes (IMVA-HB\/IDMIT\/UMR1184), Universit\u00e9 Paris-Saclay, Le Kremlin Bic\u00eatre, France"},{"name":"D\u00e9partement de Rhumatologie, H\u00f4pital Bic\u00eatre, AP-HP, INSERM UMR1184, Universit\u00e9 Paris Saclay, Le Kremlin Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9212-0859","authenticated-orcid":false,"given":"Laurent","family":"Gilardin","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine interne, H\u00f4pital Jean Verdier, AP-HP. 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Sorbonne Universit\u00e9, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6809-0733","authenticated-orcid":false,"given":"Ga\u00e9tane","family":"Nocturne","sequence":"additional","affiliation":[{"name":"Inserm, CEA, Immunologie des maladies virales, auto-immunes, h\u00e9matologiques et bact\u00e9riennes (IMVA-HB\/IDMIT\/UMR1184), Universit\u00e9 Paris-Saclay, Le Kremlin Bic\u00eatre, France"},{"name":"D\u00e9partement de Rhumatologie, H\u00f4pital Bic\u00eatre, AP-HP, INSERM UMR1184, Universit\u00e9 Paris Saclay, Le Kremlin Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ra\u00efda","family":"Bouzid","sequence":"additional","affiliation":[{"name":"INSERM UMRS-1138, Centre de Recherche des Cordeliers, CNRS, Sorbonne Universit\u00e9, Universit\u00e9 Paris Cit\u00e9, Paris, France"},{"name":"Centre National de R\u00e9f\u00e9rence des Microangiopathies Thrombotiques (CNR-MAT), H\u00f4pital Saint-Antoine, AP-HP.6. 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Sorbonne Universit\u00e9, Paris, France"},{"name":"Service d'H\u00e9matologie, H\u00f4pital Saint-Antoine, AP-HP.6. 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