{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T10:49:52Z","timestamp":1782125392736,"version":"3.54.5"},"reference-count":49,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2025,5,29]],"date-time":"2025-05-29T00:00:00Z","timestamp":1748476800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2025,5,29]],"date-time":"2025-05-29T00:00:00Z","timestamp":1748476800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100016239","name":"University of Occupational and Environmental Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100016239","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J of Rheum Dis"],"published-print":{"date-parts":[[2025,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>This study examined the efficacy and safety of Rituximab (RTX) treatment in connective tissue disease (CTD)\u2010associated thrombocytopenic purpura (TTP) and thrombotic microangiopathy (TMA), using historical controls as comparators.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients who were admitted to our department from March 1, 2013 to March 31, 2021, and diagnosed with CTD\u2010associated TTP\/TMA refractory to plasma exchange were included in the study. A patient with treatment\u2010resistant disease was treated with RTX in addition to high\u2010dose glucocorticoid (GC) therapy (GC\u2009+\u2009RTX). As historical controls, we selected patients with CTD\u2010associated TTP\/TMA who were admitted to our center and treated with GC and immunosuppressants (IS) such as cyclophosphamide. The primary endpoint was the survival rate 52\u2009weeks after the start of treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Fifteen patients were enrolled in the study (GC\u2009+\u2009RTX). As a control group, 11 patients were enrolled in the same manner (GC\u2009+\u2009IS). There were no significant differences in age or sex or laboratory tests between the two groups. The primary endpoint of survival rate was significantly higher in the GC\u2009+\u2009RTX group than in the GC\u2009+\u2009IS group. In the immunophenotyping analysis before treatment, among all subsets of immune cells, only plasmocytes were significantly elevated in TTP patients compared to healthy controls. Plasmocytes correlated with serum markers, suggesting increased B cell differentiation, which was markedly decreased after RTX treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In CTD\u2010associated TTP\/TMA, B cells may affect pathology, and adding RTX to plasma exchange and GC therapy may be worth considering.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/1756-185x.70292","type":"journal-article","created":{"date-parts":[[2025,5,30]],"date-time":"2025-05-30T01:10:02Z","timestamp":1748567402000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Efficacy and Safety of Rituximab in Connective Tissue Disease\u2010Associated Thrombotic Thrombocytopenic Purpura\/Thrombotic Microangiopathy"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2265-4104","authenticated-orcid":false,"given":"Naoaki","family":"Ohkubo","sequence":"first","affiliation":[{"name":"The First Department of Internal Medicine, School of Medicine University of Occupational and Environmental Health  Kitakyushu, Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shingo","family":"Nakayamada","sequence":"additional","affiliation":[{"name":"The First Department of Internal Medicine, School of Medicine University of Occupational and Environmental Health  Kitakyushu, Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shunsuke","family":"Fukuyo","sequence":"additional","affiliation":[{"name":"The First Department of Internal Medicine, School of Medicine University of Occupational and Environmental Health  Kitakyushu, Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yusuke","family":"Miyazaki","sequence":"additional","affiliation":[{"name":"The First Department of Internal Medicine, School of Medicine University of Occupational and Environmental Health  Kitakyushu, Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshino","family":"Inoue","sequence":"additional","affiliation":[{"name":"The First Department of Internal Medicine, School of Medicine University of Occupational and Environmental Health  Kitakyushu, Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroaki","family":"Tanaka","sequence":"additional","affiliation":[{"name":"The First Department of Internal Medicine, School of Medicine University of Occupational and Environmental Health  Kitakyushu, Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuyuki","family":"Todoroki","sequence":"additional","affiliation":[{"name":"The First Department of Internal Medicine, School of Medicine University of Occupational and Environmental Health  Kitakyushu, Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0807-7139","authenticated-orcid":false,"given":"Yoshiya","family":"Tanaka","sequence":"additional","affiliation":[{"name":"The First Department of Internal Medicine, School of Medicine University of Occupational and Environmental Health  Kitakyushu, Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,5,29]]},"reference":[{"key":"e_1_2_11_2_1","first-page":"21","article-title":"Hyaline Thrombosis of the Terminal Arterioles and Capillaries: A Hitherto Undescribed Disease","volume":"24","author":"Moschcowitz E.","year":"1924","journal-title":"Proceedings of the New York Pathological Society"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1312353"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/1756-185X.13817"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keq395"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/asheducation\u20102004.1.407"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1191\/0961203302lu224oa"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncprheum0511"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13075-020-02215-x"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365\u20102141.2012.09167.x"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12185-016-2019-x"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-10-559211"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11239-012-0723-9"},{"key":"e_1_2_11_14_1","first-page":"1194","article-title":"Rituximab Treatment of Thrombotic Thrombocytopenic Purpura in the Setting of Connective Tissue Disease","volume":"33","author":"Niewold T. 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