{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,10]],"date-time":"2026-01-10T05:44:22Z","timestamp":1768023862306,"version":"3.49.0"},"reference-count":31,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2022,10,20]],"date-time":"2022-10-20T00:00:00Z","timestamp":1666224000000},"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":[[2023,1,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>We describe the case of a 78-year-old man presenting with multiple oedematous erythemas, fever, and arthralgia who subsequently developed neutrophil infiltration into the cartilage of the bilateral auricularis, consistent with relapsing polychondritis. A skin biopsy of the erythema on his right arm showed dense neutrophilic infiltration into the dermis, while a bone marrow aspirate revealed myelodysplastic syndromes with characteristic vacuoles in myeloid precursor cells. Although the patient achieved remission with high-dose oral prednisolone, the inflammatory symptoms relapsed, and he was resistant to colchicine and cyclosporine. The patient spontaneously developed left leg oedema and high-output cardiac failure caused by an arteriovenous fistula with a common iliac artery aneurysm. We successfully performed a two-stage surgery using internal iliac artery coil embolisation and endovascular aortic repair of the iliac aneurysm. We assumed the patient was suffering from large-vessel vasculitis such as giant cell arteritis or Takayasu\u2019s arteritis. We treated him with tocilizumab in addition to prednisolone, and the febrile events and elevated C-reactive protein levels improved. One year later, sequencing of ubiquitylation-initiating E1 enzyme using peripheral blood leucocytes revealed somatic variants (c.121A&amp;gt;C p.Met41Leu), confirming the diagnosis of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome. This case suggests that arteriovenous fistula could be a complication of VEXAS syndrome with large-vessel vasculitis, and adequate surgical intervention and prompt diagnosis are essential for rescue. Although arteriovenous fistula is a rare complication of VEXAS syndrome, physicians should be aware of this complication to ensure prompt diagnosis and timely surgical intervention.<\/jats:p>","DOI":"10.1093\/mrcr\/rxac082","type":"journal-article","created":{"date-parts":[[2022,10,20]],"date-time":"2022-10-20T18:02:44Z","timestamp":1666288964000},"page":"327-333","source":"Crossref","is-referenced-by-count":13,"title":["Acute heart failure due to left common iliac arteriovenous fistula: A case of VEXAS syndrome"],"prefix":"10.1093","volume":"7","author":[{"given":"Hiroki","family":"Yamaguchi","sequence":"first","affiliation":[{"name":"Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences , Niigata, Japan"},{"name":"Department of Nephrology and Rheumatology, Niigata City General Hospital , Niigata, Japan"},{"name":"Department of Pediatrics, Child Health Research Center, University of Virginia School of Medicine , Charlottesville, VA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0384-8678","authenticated-orcid":false,"given":"Daisuke","family":"Kobayashi","sequence":"additional","affiliation":[{"name":"Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences , Niigata, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gen","family":"Nakamura","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Rheumatology, Niigata City General Hospital , Niigata, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryo","family":"Aida","sequence":"additional","affiliation":[{"name":"Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences , Niigata, 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Niigata, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naomi","family":"Tsuchida","sequence":"additional","affiliation":[{"name":"Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine , Kanagawa, Japan"},{"name":"Department of Human Genetics, Yokohama City University Graduate School of Medicine , Kanagawa, Japan"},{"name":"Department of Rare Disease Genomics, Yokohama City University Hospital , Kanagawa, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuri","family":"Uchiyama","sequence":"additional","affiliation":[{"name":"Department of Human Genetics, Yokohama City University Graduate School of Medicine , Kanagawa, Japan"},{"name":"Department of Rare Disease Genomics, Yokohama City University Hospital , Kanagawa, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayaka","family":"Maeda","sequence":"additional","affiliation":[{"name":"Department of Stem Cell and Immune 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Niigata, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eriko","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences , Niigata, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayako","family":"Wakamatsu","sequence":"additional","affiliation":[{"name":"Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences , Niigata, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ichiei","family":"Narita","sequence":"additional","affiliation":[{"name":"Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences , Niigata, 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