{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,3]],"date-time":"2025-08-03T22:52:35Z","timestamp":1754261555533,"version":"3.41.2"},"reference-count":14,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2024,9,8]],"date-time":"2024-09-08T00:00:00Z","timestamp":1725753600000},"content-version":"vor","delay-in-days":7,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Respirology Case Reports"],"published-print":{"date-parts":[[2024,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>VEXAS (Vacuoles, E1 enzyme, X\u2010linked, Autoinflammatory, Somatic) syndrome is a rare and recently identified disease resulting from a somatic mutation in the X\u2010linked UBA1 gene in cells of myeloid lineage. It can present in a myriad of ways with the potential to affect various organ systems, including the lungs. VEXAS is usually steroid responsive, but no strong data exists for the use of a steroid\u2010sparing agent. There is limited emerging evidence for haematopoietic stem cell transplantation in a select number of cases. Regardless, prognosis for this condition is poor and a treatment algorithm remains a priority. Herein, we present a case of VEXAS that came to attention with discovery of a relatively asymptomatic interstitial lung disease and led to recurrent febrile episodes with evolving multi\u2010organ involvement.<\/jats:p>","DOI":"10.1002\/rcr2.70020","type":"journal-article","created":{"date-parts":[[2024,9,9]],"date-time":"2024-09-09T03:50:10Z","timestamp":1725853810000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["A vexing case of a 73\u2010year\u2010old man with fevers, orbital cellulitis, and asymptomatic interstitial lung disease"],"prefix":"10.1002","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3889-2249","authenticated-orcid":false,"given":"Sushil","family":"Agwan","sequence":"first","affiliation":[{"name":"Department of Thoracic Medicine The Prince Charles Hospital  Chermside Queensland Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lai\u2010Ying","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Thoracic Medicine The Prince Charles Hospital  Chermside Queensland Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Baker","sequence":"additional","affiliation":[{"name":"Department of Thoracic Medicine The Prince Charles Hospital  Chermside Queensland Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Lane","sequence":"additional","affiliation":[{"name":"Faculty of Medicine The University of Queensland  Herston Queensland Australia"},{"name":"Department of Clinical Immunology and Allergy Royal Brisbane and Women's Hospital  Herston Queensland Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Godbolt","sequence":"additional","affiliation":[{"name":"Anatomical Pathology The Prince Charles Hospital  Chermside Queensland Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5254-4144","authenticated-orcid":false,"given":"John A.","family":"Mackintosh","sequence":"additional","affiliation":[{"name":"Department of Thoracic Medicine The Prince Charles Hospital  Chermside Queensland Australia"},{"name":"Faculty of Medicine The University of Queensland  Herston Queensland 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