{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,15]],"date-time":"2026-08-15T05:35:33Z","timestamp":1786772133837,"version":"build-2736575974"},"reference-count":11,"publisher":"MDPI AG","issue":"14","license":[{"start":{"date-parts":[[2026,7,21]],"date-time":"2026-07-21T00:00:00Z","timestamp":1784592000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: The clinical presentation of nasal mucosal necrosis, an exceedingly rare manifestation of secondary antiphospholipid syndrome (APS) in patients with systemic lupus erythematosus, often mimics invasive fungal sinusitis (IFS), causing diagnostic challenges and potentially unnecessary surgical interventions. Here, we report a unique case of nasal necrosis exacerbation by thrombopoietin receptor agonist (TPO-RA) therapy. Case Description: A 54-year-old woman with systemic lupus erythematosus and APS developed rapid nasal mucosal necrosis after receiving TPO-RA. An initial clinical suspicion of IFS led to emergency surgical debridement, and histopathology revealed fibrin-rich intraluminal microthrombi without fungal elements. After replacing eltrombopag with fostamatinib (a Syk inhibitor), the nasal lesions improved markedly, with complete mucosal healing. Conclusions: Nasal mucosal necrosis, a rare but serious symptom of secondary APS, requires careful differential diagnosis from IFS or other necrotizing diseases. APS-related vasculopathy should be considered following TPO-RA therapy, particularly if repeat debridement fails to identify an infectious cause.<\/jats:p>","DOI":"10.3390\/jcm15145711","type":"journal-article","created":{"date-parts":[[2026,7,21]],"date-time":"2026-07-21T10:35:52Z","timestamp":1784630152000},"page":"5711","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Nasal Mucosal Necrosis in a Patient with Systemic Lupus Erythematosus and Antiphospholipid Syndrome: A Case Report and Literature Review"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9288-5368","authenticated-orcid":false,"given":"Dong Hoon","family":"Lee","sequence":"first","affiliation":[{"name":"Department of Otolaryngology-Head and Neck Surgery, Chonnam National University Medical School, Chonnam National University Hwasun Hospital, Hwasun 58128, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ki-Jeong","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Chonnam National University Medical School, Chonnam National University Hospital, Gwangju 61469, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seo-Yeon","family":"Ahn","sequence":"additional","affiliation":[{"name":"Department of Hematology-Oncology, Chonnam National University Medical School, Chonnam National University Hwasun Hospital, Hwasun 58128, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0551-5954","authenticated-orcid":false,"given":"Joo Yeon","family":"Koo","sequence":"additional","affiliation":[{"name":"Department of Pathology, Chonnam National University Medical School, Chonnam National University Hwasun Hospital, Hwasun 58128, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sang Chul","family":"Lim","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology-Head and Neck Surgery, Chonnam National University Medical School, Chonnam National University Hwasun Hospital, Hwasun 58128, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,7,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"298","DOI":"10.1001\/archoto.2011.7","article-title":"Severe nasal presentation of antiphospholipid syndrome","volume":"137","author":"Kim","year":"2011","journal-title":"Arch. Otolaryngol. Head. Neck Surg."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1197","DOI":"10.1017\/S0022215107008754","article-title":"Nasal septal perforation and antiphospholipid syndrome (Hughes syndrome)","volume":"121","author":"Banerjee","year":"2007","journal-title":"J. Laryngol. Otol."},{"key":"ref_3","first-page":"000534","article-title":"Nasal Skin Necrosis: A Very Rare Manifestation of Antiphospholipid Syndrome","volume":"4","author":"Ourique","year":"2017","journal-title":"Eur. J. Case Rep. Intern Med."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1972","DOI":"10.1002\/ajh.70052","article-title":"Efficacy and Thrombotic Risk of Thrombopoietin Receptor Agonists for Immune Thrombocytopenia Secondary to Systemic Lupus and Antiphospholipid Syndrome: French Experience With 80 Patients","volume":"100","author":"Marques","year":"2025","journal-title":"Am. J. Hematol."},{"key":"ref_5","first-page":"e62804","article-title":"Severe Thrombocytopenia Secondary to Systemic Lupus Erythematosus With Antiphospholipid Antibodies in a Middle-Aged Woman","volume":"16","author":"Matsuura","year":"2024","journal-title":"Cureus"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1019","DOI":"10.1002\/art.10187","article-title":"Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1000 patients","volume":"46","author":"Cervera","year":"2002","journal-title":"Arthritis Rheum."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1097\/BOR.0000000000000932","article-title":"Antiphospholipid syndrome management: A 2023 update and practical algorithm-based approach","volume":"35","author":"Ambati","year":"2023","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"2567","DOI":"10.1016\/j.jtha.2026.04.002","article-title":"Risk of thrombosis in adults with primary immune thrombocytopenia treated with eltrombopag vs romiplostim: A target trial emulation in the French Adult Immune Thrombocytopenia (FAITH) cohort","volume":"24","author":"Zordan","year":"2026","journal-title":"J. Thromb. Haemost."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1002\/ajh.70194","article-title":"Et le groupe des investigateurs de CARMEN-France. Real-World Effectiveness and Safety of Fostamatinib in Difficult-to-Treat Immune Thrombocytopenia Patients. A Prospective, Multicenter Registry in France","volume":"101","author":"Moulis","year":"2026","journal-title":"Am. J. Hematol."},{"key":"ref_10","doi-asserted-by":"crossref","unstructured":"Dziedzic, R., W\u0119giel, M., Siwiec-Ko\u017alik, A., Spa\u0142kowska, M., Zar\u0119ba, L., Bazan-Socha, S., Korkosz, M., and Kosa\u0142ka-W\u0119giel, J. (2025). Thromboembolic Episodes in Patients with Systemic Lupus Erythematosus Without Atrial Fibrillation\/Atrial Flutter Are Related to the Presence of at Least 3 Points in the CHA2DS2-VA Score: A Comprehensive Retrospective Analysis of 787 Patients. J. Clin. Med., 14.","DOI":"10.3390\/jcm14113920"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"161","DOI":"10.5152\/eurjrheum.2018.18111","article-title":"Nasal septal perforation in systemic lupus erythematosus","volume":"6","author":"Thomas","year":"2018","journal-title":"Eur. J. Rheumatol."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/14\/5711\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,21]],"date-time":"2026-07-21T11:05:44Z","timestamp":1784631944000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/14\/5711"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7,21]]},"references-count":11,"journal-issue":{"issue":"14","published-online":{"date-parts":[[2026,7]]}},"alternative-id":["jcm15145711"],"URL":"https:\/\/doi.org\/10.3390\/jcm15145711","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7,21]]}}}