{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T15:01:11Z","timestamp":1790694071611,"version":"4.1.0"},"reference-count":16,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T00:00:00Z","timestamp":1789516800000},"content-version":"vor","delay-in-days":15,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pathology International"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Vacuoles, E1 enzyme, X\u2010linked, Autoinflammatory, Somatic (VEXAS) syndrome is a disease concept characterized by\n                    <jats:italic>UBA1<\/jats:italic>\n                    mutation that was first proposed in 2020. We report an autopsy case of VEXAS syndrome that was retrospectively diagnosed 11 years later through genetic analysis of formalin\u2010fixed, paraffin\u2010embedded (FFPE) bone marrow tissue. The patient was a 65\u2010year\u2010old male with hepatitis C who presented with general fatigue followed by fever 2 weeks later. Shortly before death, phlebitis of the extremities and marked leukocytosis became evident. The patient died within 3 weeks of symptom onset. Autopsy revealed granulocytic vasculitis involving small\u2010 to medium\u2010sized arteries and phlebitis in various organs, including the lungs, kidneys, liver, and lymph nodes. Concomitant myelodysplastic syndrome (MDS) was confirmed, leading to a diagnosis of MDS\u2010associated vasculitis. Sanger sequencing of FFPE specimens 11 years later revealed a\n                    <jats:italic>UBA1<\/jats:italic>\n                    mutation (p. Met41Val), confirming VEXAS syndrome. Although VEXAS syndrome is typically characterized by chronic, treatment\u2010refractory inflammation and poor prognosis, autopsy reports are rare. Herein, we report an autopsy case of VEXAS syndrome associated with MDS and systemic vasculitis that presented with a fulminant clinical course.\n                  <\/jats:p>","DOI":"10.1111\/pin.70180","type":"journal-article","created":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T14:11:33Z","timestamp":1789567893000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Acute Fulminant Systemic Granulocytic Vasculitis Involving Arteries and Veins in Vacuoles, E1 Enzyme, X\u2010Linked, Autoinflammatory, Somatic (VEXAS) Syndrome: An Autopsy Case Report"],"prefix":"10.1111","volume":"76","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-7792-1969","authenticated-orcid":false,"given":"Kayo","family":"Ueda","sequence":"first","affiliation":[{"name":"Department of Pathology Osaka Habikino Medical Center Osaka Japan"},{"name":"Department of Pathology Yodogawa Christian Hospital Osaka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0575-6507","authenticated-orcid":false,"given":"Masakazu","family":"Fujimoto","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology Kyoto University Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yumi","family":"Shiroishi","sequence":"additional","affiliation":[{"name":"Cell and Genetic Laboratory Kobe City Medical Center General Hospital Kobe Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masafumi","family":"Ito","sequence":"additional","affiliation":[{"name":"Department of Pathology Japanese Red Cross Aichi Medical Center, Nagoya Daiichi Hospital Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahiko","family":"Osawa","sequence":"additional","affiliation":[{"name":"Department of Pathology Ishikiriseiki Hospital Higashiosaka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mayumi","family":"Inaba","sequence":"additional","affiliation":[{"name":"Department of Pathology Yodogawa Christian Hospital Osaka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,9,16]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2026834"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2022.24836"},{"key":"e_1_2_10_4_1","volume-title":"World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues","author":"Swerdlow S. 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