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We present a patient with antiphospholipid syndrome who developed multiorgan complications and was diagnosed with probable CAPS. Diagnosis was complicated due to thrombotic risk and the heterogeneous nature of CAPS, as well as his unresponsiveness to first-line therapy consisting of anticoagulation and corticosteroids. His treatment was then escalated with plasma exchange, rituximab and cyclophosphamide, to which he responded and improved clinically.<\/jats:p>","DOI":"10.1136\/bcr-2024-263766","type":"journal-article","created":{"date-parts":[[2025,6,8]],"date-time":"2025-06-08T15:40:15Z","timestamp":1749397215000},"page":"e263766","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Complex presentation of probable catastrophic antiphospholipid syndrome: diagnostic dilemmas and treatment strategies"],"prefix":"10.1136","volume":"18","author":[{"given":"Ranya","family":"Khaleel","sequence":"first","affiliation":[{"name":"Melbourne Medical School, The University of Melbourne, Melbourne, Victoria, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2728-4299","authenticated-orcid":false,"given":"Hannah","family":"Wallace","sequence":"additional","affiliation":[{"name":"Melbourne Medical School, The University of Melbourne, Melbourne, Victoria, Australia"},{"name":"Nephrology, Western Health, St Albans, Victoria, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2025,6,8]]},"reference":[{"key":"2025082722011940000_18.6.e263766.1","first-page":"1675","article-title":"Antiphospholipid syndrome: an overview","volume":"168","author":"Hanly","year":"2003","journal-title":"CMAJ"},{"key":"2025082722011940000_18.6.e263766.2","doi-asserted-by":"publisher","DOI":"10.1007\/s11926-021-01038-2"},{"key":"2025082722011940000_18.6.e263766.3","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1016\/j.rcreu.2021.02.004","article-title":"Catastrophic antiphospholipid syndrome","volume":"28","author":"Rodriguez-Pint\u00f3","year":"2021","journal-title":"Revista Colombiana de Reumatolog\u00eda"},{"key":"2025082722011940000_18.6.e263766.4","first-page":"530","article-title":"Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines","volume":"12","author":"Asherson","year":"2003","journal-title":"Lupus (Los Angel)"},{"key":"2025082722011940000_18.6.e263766.5","doi-asserted-by":"publisher","DOI":"10.1097\/CM9.0000000000000705"},{"key":"2025082722011940000_18.6.e263766.6","doi-asserted-by":"crossref","first-page":"668","DOI":"10.3390\/ijms25010668","article-title":"Catastrophic Antiphospholipid Syndrome","volume":"25","author":"Bitsadze","year":"2024","journal-title":"Int J Mol Sci"},{"key":"2025082722011940000_18.6.e263766.7","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V97.4.844"},{"key":"2025082722011940000_18.6.e263766.8","unstructured":"RITUXAN dosing and administration. 2024."},{"key":"2025082722011940000_18.6.e263766.9","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3390\/immuno4010001","article-title":"Catastrophic Antiphospholipid Syndrome: A Review","volume":"4","author":"Siniscalchi","year":"2024","journal-title":"Immuno"},{"key":"2025082722011940000_18.6.e263766.10","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2016.09.010"},{"key":"2025082722011940000_18.6.e263766.11","doi-asserted-by":"crossref","first-page":"1656","DOI":"10.1111\/jth.14192","article-title":"McMaster RARE-Bestpractices clinical practice guideline on diagnosis and management of the catastrophic antiphospholipid syndrome","volume":"16","author":"Legault","year":"2018","journal-title":"J Thromb Haemost"},{"key":"2025082722011940000_18.6.e263766.12","first-page":"412","article-title":"Catastrophic antiphospholipid syndrome (CAPS): update from the 'CAPS Registry'","volume":"19","author":"Cervera","year":"2010","journal-title":"Lupus (Los Angel)"},{"key":"2025082722011940000_18.6.e263766.13","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2010.08.005"},{"key":"2025082722011940000_18.6.e263766.14","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/key082"},{"key":"2025082722011940000_18.6.e263766.15","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1016\/j.autrev.2015.11.009","article-title":"Intravenous immunoglobulins and antiphospholipid syndrome: How, when and why? 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