{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T07:24:17Z","timestamp":1786951457287,"version":"build-2736575974"},"reference-count":13,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,8,16]],"date-time":"2026-08-16T00:00:00Z","timestamp":1786838400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,8,16]],"date-time":"2026-08-16T00:00:00Z","timestamp":1786838400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis Care & Research"],"abstract":"\n Objective<\/jats:title>\n Vacuoles, E1 enzyme, X\u2010linked, autoinflammatory, somatic syndrome (VEXAS) syndrome is characterized by a complex spectrum of inflammatory and hematologic manifestations. Clinical research to identify effective therapies is urgently needed but is hindered by the lack of validated outcome measures. A VEXAS\u2010specific disease activity index (DAI) is an essential tool for reliably capturing changes in disease activity over time, thereby supporting clinical research and informing patient care in this emerging disease. This study aimed to develop a comprehensive DAI to assess disease\u2010related inflammation across affected organ systems in patients with VEXAS syndrome.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n Inflammatory manifestations of VEXAS were previously identified through a systematic literature review and expert input. A multinational expert advisory committee developed the VEXAS\u2010DAI using modified Delphi methodology until consensus (defined as \u226575% concurrence) was reached. Item grading was adapted from the Common Terminology Criteria for Adverse Events. Scoring criteria were developed based on 158 test cases using the Physician Global Assessment of Inflammation.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n Consensus on the VEXAS\u2010DAI was achieved after four Delphi rounds. The index assesses inflammatory activity at the time of evaluation across 12 domains: inflammatory rash, chondritis, periorbital, ophthalmologic, joint, pulmonary, cardiovascular, genitourinary, neurologic, oral and gastrointestinal, renal, and constitutional symptoms. The total score ranges from 0 to 40.<\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n The VEXAS\u2010DAI is a novel instrument designed to quantify active inflammation over time in patients with VEXAS. Prospective validation within a clinical trial setting is ongoing.<\/jats:p>\n \n \n \n image<\/jats:alt-text>\n <\/jats:graphic>\n <\/jats:boxed-text>\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/acr.80117","type":"journal-article","created":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T11:45:12Z","timestamp":1783943112000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Development of a Disease Activity Index for the Assessment of\n VEXAS<\/scp>\n Syndrome (VEXAS\u2010DAI)"],"prefix":"10.1002","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6441-1915","authenticated-orcid":false,"given":"Kevin","family":"Byram","sequence":"first","affiliation":[{"name":"Vanderbilt University Medical Center Nashville Tennessee"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4243-235X","authenticated-orcid":false,"given":"He\u0159man","family":"Mann","sequence":"additional","affiliation":[{"name":"Institute of Rheumatology and Department of Rheumatology, 1st Faculty of Medicine Charles University Prague Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6017-2264","authenticated-orcid":false,"given":"Danielle","family":"Hammond","sequence":"additional","affiliation":[{"name":"The University of Texas MD Anderson Cancer Center Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8150-4088","authenticated-orcid":false,"given":"Onima","family":"Chowdhury","sequence":"additional","affiliation":[{"name":"Oxford University Hospitals\u2019 NHS Foundation Trust Oxford UK"},{"name":"Weatherall Institute of Molecular Medicine University of Oxford Oxford UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7910-0554","authenticated-orcid":false,"given":"Sinisa","family":"Savic","sequence":"additional","affiliation":[{"name":"University of Leeds, Leeds Institute of Rheumatic and Musculoskeletal Medicine Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9488-661X","authenticated-orcid":false,"given":"Yohei","family":"Kirino","sequence":"additional","affiliation":[{"name":"Yokohama City University Graduate School of Medicine Yokohama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6829-5544","authenticated-orcid":false,"given":"Carmelo","family":"Gurnari","sequence":"additional","affiliation":[{"name":"Department of Biomedicine and Prevention University of Rome Tor Vergata Rome Italy"},{"name":"Translational Hematology and Oncology Research Department Taussig Cancer Center, Cleveland Clinic Cleveland Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1682-8657","authenticated-orcid":false,"given":"Ma\u00ebl","family":"Heiblig","sequence":"additional","affiliation":[{"name":"Lyon\u2010Sud Hospital, Hospices Civils de Lyon Paris France"},{"name":"Universit\u00e9 Claude Bernard Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6891-9238","authenticated-orcid":false,"given":"Thibault","family":"Comont","sequence":"additional","affiliation":[{"name":"University Toulouse, CHU Toulouse Toulouse France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2849-3049","authenticated-orcid":false,"given":"Ars\u00e8ne","family":"Mekinian","sequence":"additional","affiliation":[{"name":"Sorbonne Universit\u00e9 \u2010 H\u00f4pital Saint\u2010Antoine AP\u2010HP Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mrinal M.","family":"Patnaik","sequence":"additional","affiliation":[{"name":"Mayo Clinic Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8726-6212","authenticated-orcid":false,"given":"Lachelle D.","family":"Weeks","sequence":"additional","affiliation":[{"name":"Dana\u2010Farber Cancer Institute Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1531-5147","authenticated-orcid":false,"given":"Gary","family":"Ho","sequence":"additional","affiliation":[{"name":"New York University Langone Health New York New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-1535-8909","authenticated-orcid":false,"given":"Adam","family":"Al\u2010Hakim","sequence":"additional","affiliation":[{"name":"University of Leeds, Leeds Institute of Rheumatic and Musculoskeletal Medicine Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott","family":"Goldberg","sequence":"additional","affiliation":[{"name":"New York University Langone Medical Center New York New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3256-3273","authenticated-orcid":false,"given":"Marcela A.","family":"Ferrada","sequence":"additional","affiliation":[{"name":"University of Maryland School of Medicine Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6668-8854","authenticated-orcid":false,"given":"Sophie","family":"Georgin\u2010Lavialle","sequence":"additional","affiliation":[{"name":"Departement Medico\u2010Universitaire 3iD, F\u00e9d\u00e9ration Hospitalo\u2010Universitaire, Centre de R\u00e9f\u00e9rence des Maladies Auto\u2010Inflammatoires et des Amyloses, European Reference Network on Rare Immunodeficiencies, Sorbonne University Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8269-9438","authenticated-orcid":false,"given":"Peter C.","family":"Grayson","sequence":"additional","affiliation":[{"name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4648-5926","authenticated-orcid":false,"given":"Emma M.","family":"Groarke","sequence":"additional","affiliation":[{"name":"National Heart, Lung, and Blood Institute, NIH Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bhavisha","family":"Patel","sequence":"additional","affiliation":[{"name":"National Heart, Lung, and Blood Institute, NIH Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3459-1534","authenticated-orcid":false,"given":"Megan","family":"Sullivan","sequence":"additional","affiliation":[{"name":"Mayo Clinic Scottsdale Arizona"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7041-8328","authenticated-orcid":false,"given":"Sarah A.","family":"Buckley","sequence":"additional","affiliation":[{"name":"Sobi Inc Waltham Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-4912-3052","authenticated-orcid":false,"given":"Bryan G.","family":"Harder","sequence":"additional","affiliation":[{"name":"Sobi Inc Waltham Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-0409-3414","authenticated-orcid":false,"given":"Raman","family":"Garcha","sequence":"additional","affiliation":[{"name":"Sobi Inc Waltham 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Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,8,16]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2026834"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.27156"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keaf293"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.43287"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2020010177"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kead240"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/ard-2024-225640"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm15041426"},{"key":"e_1_2_8_10_1","unstructured":"A phase II study of allogeneic hematopoietic stem cell transplant for subjects with VEXAS (vacuoles E1 enzyme X\u2010linked autoinflammatory somatic) syndrome.ClinicalTrials.gov\u00a0identifier: NCT05027945. Updated July 10 2026. Accessed August 5 2026.https:\/\/clinicaltrials.gov\/study\/NCT05027945."},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-024-07160-7"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2023012478"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keae530"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keaf624"}],"container-title":["Arthritis Care & Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acr.80117","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/acr.80117","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acr.80117","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T06:31:23Z","timestamp":1786948283000},"score":1,"resource":{"primary":{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/10.1002\/acr.80117"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,16]]},"references-count":13,"alternative-id":["10.1002\/acr.80117"],"URL":"https:\/\/doi.org\/10.1002\/acr.80117","archive":["Portico"],"relation":{},"ISSN":["2151-464X","2151-4658"],"issn-type":[{"value":"2151-464X","type":"print"},{"value":"2151-4658","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,16]]},"assertion":[{"value":"2026-03-05","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-06-23","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-08-16","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"acr.80117"}}