{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,23]],"date-time":"2026-07-23T02:02:56Z","timestamp":1784772176057,"version":"3.55.0"},"reference-count":16,"publisher":"BMJ","issue":"8","funder":[{"DOI":"10.13039\/100012921","name":"Royal College of Surgeons in Ireland","doi-asserted-by":"publisher","award":["RCSI Surgical Travel Grant"],"award-info":[{"award-number":["RCSI Surgical Travel Grant"]}],"id":[{"id":"10.13039\/100012921","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Br J Ophthalmol"],"accepted":{"date-parts":[[2026,1,7]]},"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Vacuoles, E1-ligase, X-linked Auto-inflammatory, Somatic (VEXAS) syndrome is a recently described multisystem inflammatory disorder. Ocular features are described, though not well known; we aimed to describe them.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This is a case series of VEXAS patients submitted by ophthalmologists from relevant specialty organisations, including the Australian and New Zealand Society of Ophthalmic Plastic Surgeons, British Oculoplastic Surgical Society and the Orbital Society. Patient consent was received.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      14 males with VEXAS syndrome and eye features were included, median age 73 years (range 49\u201378). The associated\n                      <jats:italic>UBA1<\/jats:italic>\n                      mutation was most commonly p.Met41Val (n=8, 57%), and most patients were Caucasian (n=11, 79%). All patients reported eyelid swelling (n=14, 100%); the next most frequent symptom was eye pain (n=10, 71%). Eye symptoms showed variable duration at presentation, most commonly 2\u20137\u2009days (n=6, 43%). Eye involvement was typically bilateral (metachronous n=5, 36%, synchronous n=2, 14%). Overall, visual acuity was normal and did not change. Severe vision loss occurred unilaterally in two patients (14%), due to orbital compartment syndrome and periorbital necrotising fasciitis. Ophthalmologist-reported clinical features included periorbital oedema, present in all cases (n=14, 100%), followed by dacryoadenitis (n=8, 57%) and orbital myositis (n=7, 50%). There were no cases of posterior segment inflammation. Death due to complications of VEXAS occurred in one patient (7%).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Orbital inflammation was a feature in all cases of VEXAS with eye involvement, and severe loss of vision occurred unilaterally in 14%. Ocular complaints in VEXAS patients should prompt urgent ophthalmic assessment.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/bjo-2025-328652","type":"journal-article","created":{"date-parts":[[2026,1,14]],"date-time":"2026-01-14T18:20:25Z","timestamp":1768414825000},"page":"936-940","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Orbital inflammation in VEXAS syndrome"],"prefix":"10.1136","volume":"110","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6914-7447","authenticated-orcid":false,"given":"Clare","family":"Quigley","sequence":"first","affiliation":[{"name":"Department of Ophthalmology, Royal Adelaide Hospital, Adelaide, South Australia, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3969-1561","authenticated-orcid":false,"given":"James","family":"Pietris","sequence":"additional","affiliation":[{"name":"The University of Queensland Faculty of Medicine, Herston, Queensland, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4530-1793","authenticated-orcid":false,"given":"Terence","family":"Ang","sequence":"additional","affiliation":[{"name":"Ophthalmology and Visual Sciences, The University of Adelaide School of Medicine, Adelaide, South Australia, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdullah","family":"Al Mater","sequence":"additional","affiliation":[{"name":"Research Excellence Center in Ophthalmology and Visual Sciences, Department of Ophthalmology, College of Medicine, King Saud University, Riyadh, Saudi Arabia"},{"name":"King Saud University Medical City, Riyadh, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Beecher","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology and Allergy, Royal Adelaide Hospital, Adelaide, South Australia, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"Oh","sequence":"additional","affiliation":[{"name":"Division of Orbital and Ophthalmic Plastic Surgery, Jules Stein Eye Institute, Los Angeles, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel Benson","family":"Rootman","sequence":"additional","affiliation":[{"name":"Ophthalmic Plastic and Reconstructive Division, Jules Stein Eye Institute, Los Angeles, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-2771-5665","authenticated-orcid":false,"given":"Nicholas James","family":"Theis","sequence":"additional","affiliation":[{"name":"Christchurch Hospital, Christchurch, New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Weatherhead","sequence":"additional","affiliation":[{"name":"Christchurch Hospital, Christchurch, New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reid","family":"Ferguson","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Waikato Hospital, Hamilton, New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen","family":"Ng","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Waikato Hospital, Hamilton, New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Verona","family":"Botha","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Waikato Hospital, Hamilton, New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ru M","family":"Ong","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Concord Repatriation General Hospital Australia, Sydney, Waikato, Australia"},{"name":"Faculty of Medicine and Health, University of New South Wales, Sydney, New South Wales, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael John","family":"Davies","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Concord Repatriation General Hospital Australia, Sydney, Waikato, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Freny","family":"Kalapesi","sequence":"additional","affiliation":[{"name":"Faculty of Medicine and Health, University of New South Wales, Sydney, New South Wales, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah F","family":"Osborne","sequence":"additional","affiliation":[{"name":"Moorfields Eye Hospital NHS Foundation Trust, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9764-474X","authenticated-orcid":false,"given":"Kaveh","family":"Vahdani","sequence":"additional","affiliation":[{"name":"Moorfields Eye Hospital NHS Foundation Trust, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie Louise Roed","family":"Rasmussen","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark"},{"name":"Department of Ophthalmology, Rigshospitalet, Copenhagen, Capital Region of Denmark, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5906-7670","authenticated-orcid":false,"given":"Steffen","family":"Heegaard","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark"},{"name":"Department of Ophthalmology, Rigshospitalet, Copenhagen, Capital Region of Denmark, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Sales-Sanz","sequence":"additional","affiliation":[{"name":"Ophthalmology, Oculoplastics and Orbital Surgery Unit, Ram\u00f3n y Cajal Hospital, Madrid, 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Australia"},{"name":"Ophthalmology and Visual Sciences, The University of Adelaide School of Medicine, Adelaide, South Australia, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2026,1,14]]},"reference":[{"key":"2026072202101934000_110.8.936.1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2026834"},{"key":"2026072202101934000_110.8.936.2","doi-asserted-by":"crossref","first-page":"e435","DOI":"10.1093\/rheumatology\/keab524","article-title":"Atypical splice-site mutations causing VEXAS syndrome","volume":"60","author":"Templ\u00e9","year":"2021","journal-title":"Rheumatology (Oxford)"},{"key":"2026072202101934000_110.8.936.3","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.20805"},{"key":"2026072202101934000_110.8.936.4","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1038\/s41584-023-00931-7","article-title":"VEXAS syndrome more common than originally 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BMC Nephrol 2024;25. doi:10.1186\/s12882-024-03454-9","DOI":"10.1186\/s12882-024-03454-9"},{"key":"2026072202101934000_110.8.936.13","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2021.05.014"},{"key":"2026072202101934000_110.8.936.14","doi-asserted-by":"crossref","first-page":"298","DOI":"10.1097\/RHU.0000000000001905","article-title":"VEXAS Syndrome-A Review of Pathophysiology, Presentation, and Prognosis","volume":"29","author":"Koster","year":"2023","journal-title":"J Clin Rheumatol"},{"key":"2026072202101934000_110.8.936.15","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1097\/IOP.0000000000002669","article-title":"Orbital Compartment Syndrome as a Novel Manifestation of VEXAS (Vacuole, E1 Enzyme, X-Linked, Autoimmune, Somatic Syndrome)","volume":"40","author":"Gupta","year":"2024","journal-title":"Ophthalmic Plast Reconstr Surg"},{"key":"2026072202101934000_110.8.936.16","doi-asserted-by":"crossref","unstructured":"Legeas C , Saucereau J , Saraux A , et al . VEXAS syndrome: A first case without any haematological abnormalities. 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