{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T12:54:09Z","timestamp":1774356849307,"version":"3.50.1"},"reference-count":21,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,1,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Systemic lupus erythematosus\u2013associated thrombotic thrombocytopenic purpura is a rare but potentially life-threatening condition that is characterised by thrombocytopenia, microangiopathic haemolytic anaemia, and multiorgan involvement. Here, we describe a refractory case of systemic lupus erythematosus\u2013associated thrombotic thrombocytopenic purpura in a 38-year-old Japanese woman who was unresponsive to plasma exchange and glucocorticoid therapy. Combination treatment with caplacizumab and rituximab leads to rapid platelet recovery, resolution of neurological symptoms, and sustained remission. Caplacizumab provided immediate inhibition of microthrombus formation, whereas rituximab targeted the underlying autoimmune process through B cell depletion. Following treatment, ADAMTS13 activity normalised, allowing for corticosteroid tapering and the maintenance of long-term remission. This case highlights the value of early combination therapy for refractory systemic lupus erythematosus\u2013associated thrombotic thrombocytopenic purpura, and it suggests that early combination therapy with caplacizumab and rituximab may act synergistically to improve outcomes.<\/jats:p>","DOI":"10.1093\/mrcr\/rxag013","type":"journal-article","created":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T12:43:26Z","timestamp":1772196206000},"source":"Crossref","is-referenced-by-count":0,"title":["Triple therapy with caplacizumab and rituximab for refractory systemic lupus erythematosus-associated thrombotic thrombocytopenic purpura achieves rapid, durable remission: A case report"],"prefix":"10.1093","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-4940-6198","authenticated-orcid":false,"given":"Manabu","family":"Honda","sequence":"first","affiliation":[{"name":"Department of Rheumatology, Shimane University Faculty of Medicine , Izumo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saaya","family":"Nakazato","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Shimane University Faculty of Medicine , Izumo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tomoko","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Shimane University Faculty of Medicine , Izumo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ikuko","family":"Takano","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Shimane University Faculty of Medicine , Izumo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mayuko","family":"Moriyama","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Shimane University Faculty of Medicine , Izumo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masahiro","family":"Kondo","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Shimane University Faculty of Medicine , Izumo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kunihiro","family":"Ichinose","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Shimane University Faculty of Medicine , Izumo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2026,3,2]]},"reference":[{"key":"2026032408041019900_ref1","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1016\/j.hoc.2007.06.003","article-title":"Thrombotic thrombocytopenic purpura: a thrombotic disorder caused by ADAMTS13 deficiency","volume":"21","author":"Tsai","year":"2007","journal-title":"Hematol Oncol Clin North Am"},{"key":"2026032408041019900_ref2","first-page":"1746","article-title":"Clinical features and predictors of survival in adult patients with thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency","volume":"25","author":"Jang","year":"2010","journal-title":"J Korean Med Sci"},{"key":"2026032408041019900_ref3","doi-asserted-by":"crossref","first-page":"824","DOI":"10.1093\/rheumatology\/keq395","article-title":"Microangiopathic haemolytic anaemia resembling thrombotic thrombocytopenic purpura in systemic lupus erythematosus: the role of ADAMTS13","volume":"50","author":"Lansigan","year":"2011","journal-title":"Rheumatology (Oxford)"},{"key":"2026032408041019900_ref4","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1007\/s10067-013-2312-5","article-title":"Clinical features and prognostic factors of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a literature review of 105 cases from 1999 to 2011","volume":"33","author":"Jiang","year":"2014","journal-title":"Clin Rheumatol"},{"key":"2026032408041019900_ref5","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1182\/blood.2020008021","article-title":"A regimen with caplacizumab, immunosuppression, and plasma exchange prevents unfavorable outcomes in immune-mediated TTP","volume":"137","author":"Coppo","year":"2021","journal-title":"Blood."},{"key":"2026032408041019900_ref6","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1056\/NEJMoa1806311","article-title":"Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura","volume":"380","author":"Scully","year":"2019","journal-title":"N Engl J Med"},{"key":"2026032408041019900_ref7","first-page":"00360","article-title":"International Society on Thrombosis and Haemostasis. 2025 focused update of the 2020 ISTH guidelines for management of thrombotic thrombocytopenic purpura","volume":"S1538-7836","author":"Zheng","year":"2025","journal-title":"J Thromb 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Nephrol Case Stud"},{"key":"2026032408041019900_ref11","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1186\/s12969-024-01049-0","article-title":"Thrombotic thrombocytopenic purpura with juvenile systemic lupus erythematosus: successful treatment with caplacizumab and rituximab","volume":"22","author":"Yokoyama","year":"2024","journal-title":"Pediatr Rheumatol Online J"},{"key":"2026032408041019900_ref12","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1159\/000514283","article-title":"An early unexpected immune thrombotic thrombocytopenic purpura relapse associated with SARS-CoV-2 infection: a case report and literature review","volume":"144","author":"Kornowski Cohen","year":"2021","journal-title":"Acta Haematol"},{"key":"2026032408041019900_ref13","doi-asserted-by":"crossref","first-page":"2496","DOI":"10.1111\/jth.15010","article-title":"ISTH guidelines for treatment of thrombotic thrombocytopenic 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of acute thrombotic thrombocytopenic purpura","volume":"137","author":"Dutt","year":"2021","journal-title":"Blood."},{"key":"2026032408041019900_ref17","doi-asserted-by":"crossref","first-page":"3085","DOI":"10.1182\/bloodadvances.2020001973","article-title":"Real-world data confirm the effectiveness of caplacizumab in acquired thrombotic thrombocytopenic purpura","volume":"4","author":"V\u00f6lker","year":"2020","journal-title":"Blood Adv"},{"key":"2026032408041019900_ref18","doi-asserted-by":"crossref","first-page":"2132","DOI":"10.1182\/bloodadvances.2022008443","article-title":"Adding caplacizumab to standard of care in thrombotic thrombocytopenic purpura: a systematic review and meta-analysis","volume":"7","author":"Djulbegovic","year":"2023","journal-title":"Blood Adv"},{"key":"2026032408041019900_ref19","first-page":"277","article-title":"Economic evaluation of caplacizumab for acquired thrombotic thrombocytopenic 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