{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T20:43:24Z","timestamp":1790109804265,"version":"4.0.1"},"reference-count":37,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2021,10,1]],"date-time":"2021-10-01T00:00:00Z","timestamp":1633046400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2021,10,1]],"date-time":"2021-10-01T00:00:00Z","timestamp":1633046400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2021,10,1]],"date-time":"2021-10-01T00:00:00Z","timestamp":1633046400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2021,10,1]],"date-time":"2021-10-01T00:00:00Z","timestamp":1633046400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2021,10,1]],"date-time":"2021-10-01T00:00:00Z","timestamp":1633046400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2021,10,1]],"date-time":"2021-10-01T00:00:00Z","timestamp":1633046400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2021,10,1]],"date-time":"2021-10-01T00:00:00Z","timestamp":1633046400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Seminars in Hematology"],"published-print":{"date-parts":[[2021,10]]},"DOI":"10.1053\/j.seminhematol.2021.10.002","type":"journal-article","created":{"date-parts":[[2021,10,11]],"date-time":"2021-10-11T11:41:48Z","timestamp":1633952508000},"page":"218-225","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":31,"title":["VEXAS within the spectrum of rheumatologic disease"],"prefix":"10.1053","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2895-6755","authenticated-orcid":false,"given":"Matthew J.","family":"Koster","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth J.","family":"Warrington","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"9","key":"10.1053\/j.seminhematol.2021.10.002_bib0001","doi-asserted-by":"crossref","first-page":"1481","DOI":"10.1517\/14656566.9.9.1481","article-title":"Intrinsic and treatment-related immune alterations in chronic lymphocytic leukaemia and their impact for clinical practice","volume":"9","author":"Dasanu","year":"2008","journal-title":"Expert Opin Pharmacother"},{"issue":"15","key":"10.1053\/j.seminhematol.2021.10.002_bib0002","doi-asserted-by":"crossref","first-page":"2369","DOI":"10.1517\/14656566.2011.591381","article-title":"Autoimmune phenomena in untreated and treated marginal zone lymphoma","volume":"12","author":"Grabska","year":"2011","journal-title":"Expert Opin Pharmacother"},{"issue":"6","key":"10.1053\/j.seminhematol.2021.10.002_bib0003","doi-asserted-by":"crossref","first-page":"1746","DOI":"10.1182\/bloodadvances.2020003193","article-title":"Rheumatologic diseases impact the risk of progression of MGUS to overt multiple myeloma","volume":"5","author":"Steiner","year":"2021","journal-title":"Blood Adv"},{"issue":"13","key":"10.1053\/j.seminhematol.2021.10.002_bib0004","doi-asserted-by":"crossref","first-page":"e25406","DOI":"10.1097\/MD.0000000000025406","article-title":"Autoimmune manifestations associated with myelodysplastic syndrome predict a poor prognosis","volume":"100","author":"Arinobu","year":"2021","journal-title":"Medicine (Baltimore)"},{"issue":"27","key":"10.1053\/j.seminhematol.2021.10.002_bib0005","doi-asserted-by":"crossref","first-page":"2628","DOI":"10.1056\/NEJMoa2026834","article-title":"Somatic mutations in UBA1 and severe adult-onset autoinflammatory disease","volume":"383","author":"Beck","year":"2020","journal-title":"N Engl J Med"},{"issue":"10","key":"10.1053\/j.seminhematol.2021.10.002_bib0006","doi-asserted-by":"crossref","first-page":"1886","DOI":"10.1002\/art.41743","article-title":"Somatic mutations in UBA1 define a distinct subset of relapsing polychondritis patients with VEXAS syndrome","volume":"73","author":"Ferrada","year":"2021","journal-title":"Arthritis Rheumatol"},{"key":"10.1053\/j.seminhematol.2021.10.002_bib0007","article-title":"Adult-onset autoinflammation caused by somatic mutations in UBA1: a Dutch case series of VEXAS patients","author":"van der Made","year":"2021","journal-title":"J Allergy Clin Immunol"},{"issue":"26","key":"10.1053\/j.seminhematol.2021.10.002_bib0008","doi-asserted-by":"crossref","first-page":"3682","DOI":"10.1182\/blood.2020010177","article-title":"Therapeutic options in Vexas syndrome: insights from a retrospective series","volume":"137","author":"Bourbon","year":"2021","journal-title":"Blood"},{"issue":"26","key":"10.1053\/j.seminhematol.2021.10.002_bib0009","doi-asserted-by":"crossref","first-page":"3676","DOI":"10.1182\/blood.2020010286","article-title":"Novel somatic mutations in UBA1 as a cause of VEXAS syndrome","volume":"137","author":"Poulter","year":"2021","journal-title":"Blood"},{"key":"10.1053\/j.seminhematol.2021.10.002_bib0010","doi-asserted-by":"crossref","DOI":"10.1136\/annrheumdis-2021-220089","article-title":"Pathogenic UBA1 variants associated with VEXAS syndrome in Japanese patients with relapsing polychondritis","author":"Tsuchida","year":"2021","journal-title":"Ann Rheum Dis"},{"issue":"26","key":"10.1053\/j.seminhematol.2021.10.002_bib0011","doi-asserted-by":"crossref","first-page":"3685","DOI":"10.1182\/blood.2021010811","article-title":"Vacuolization of hematopoietic precursors: an enigma with multiple etiologies","volume":"137","author":"Gurnari","year":"2021","journal-title":"Blood"},{"key":"10.1053\/j.seminhematol.2021.10.002_bib0012","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2021.678927","article-title":"Case report: vexas syndrome: from mild symptoms to life-threatening macrophage activation syndrome","volume":"12","author":"Staels","year":"2021","journal-title":"Front Immunol"},{"issue":"9","key":"10.1053\/j.seminhematol.2021.10.002_bib0013","doi-asserted-by":"crossref","first-page":"e304","DOI":"10.1093\/rheumatology\/keab200","article-title":"A case of VEXAS syndrome manifesting as Kikuchi-Fujimoto disease, relapsing polychondritis, venous thromboembolism and macrocytic anaemia","volume":"60","author":"Lee","year":"2021","journal-title":"Rheumatology (Oxford)"},{"issue":"10","key":"10.1053\/j.seminhematol.2021.10.002_bib0014","doi-asserted-by":"crossref","first-page":"1896","DOI":"10.1002\/art.41775","article-title":"Extensive multiple organ involvement in VEXAS syndrome","volume":"73","author":"Takahashi","year":"2021","journal-title":"Arthritis Rheumatol"},{"issue":"4","key":"10.1053\/j.seminhematol.2021.10.002_bib0015","article-title":"The VEXAS syndrome: uncontrolled inflammation and macrocytic anaemia in a 77-year-old male patient","volume":"8","author":"Himmelmann","year":"2021","journal-title":"Eur J Case Rep Intern Med"},{"key":"10.1053\/j.seminhematol.2021.10.002_bib0016","doi-asserted-by":"crossref","DOI":"10.1093\/rheumatology\/keab349","article-title":"Case of polychondritis-onset refractory organising pneumonia with cytopaenia diagnosed as VEXAS syndrome: the disease course of seven years","author":"Sakuma","year":"2021","journal-title":"Rheumatology (Oxford)"},{"key":"10.1053\/j.seminhematol.2021.10.002_bib0017","doi-asserted-by":"crossref","DOI":"10.7326\/L21-0023","article-title":"Severe joint involvement in VEXAS syndrome: a case report","author":"Lacombe","year":"2021","journal-title":"Ann Intern Med"},{"key":"10.1053\/j.seminhematol.2021.10.002_bib0018","doi-asserted-by":"crossref","DOI":"10.1093\/rheumatology\/keab211","article-title":"VEXAS syndrome in a patient with previous spondyloarthritis with favorable response to intravenous immunoglobulin anti-IL17 therapy","author":"Magnol","year":"2021","journal-title":"Rheumatology (Oxford)"},{"key":"10.1053\/j.seminhematol.2021.10.002_bib0019","doi-asserted-by":"crossref","DOI":"10.1093\/rheumatology\/keab225","article-title":"VEXAS syndrome: still expanding the clinical phenotype","author":"Oganesyan","year":"2021","journal-title":"Rheumatology (Oxford)"},{"key":"10.1053\/j.seminhematol.2021.10.002_bib0020","doi-asserted-by":"crossref","DOI":"10.1093\/rheumatology\/keab392","article-title":"VEXAS syndrome in a woman","author":"Barba","year":"2021","journal-title":"Rheumatology (Oxford)"},{"issue":"1","key":"10.1053\/j.seminhematol.2021.10.002_bib0021","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1186\/s40164-021-00217-2","article-title":"VEXAS syndrome in myelodysplastic syndrome with autoimmune disorder","volume":"10","author":"Huang","year":"2021","journal-title":"Exp Hematol Oncol"},{"key":"10.1053\/j.seminhematol.2021.10.002_bib0022","doi-asserted-by":"crossref","DOI":"10.1111\/bjh.17381","article-title":"Characteristic vacuolisation of granulocytic and erythroid precursors associated with VEXAS syndrome","author":"Rieu","year":"2021","journal-title":"Br J Haematol"},{"key":"10.1053\/j.seminhematol.2021.10.002_bib0023","doi-asserted-by":"crossref","DOI":"10.3899\/jrheum.210149","article-title":"Somatic mutation in UBA1 and ANCA-associated vasculitis","author":"Ross","year":"2021","journal-title":"J Rheumatol"},{"key":"10.1053\/j.seminhematol.2021.10.002_bib0024","doi-asserted-by":"crossref","DOI":"10.1007\/s10875-021-01070-y","article-title":"A case of VEXAS syndrome complicated by hemophagocytic lymphohistiocytosis","author":"Grey","year":"2021","journal-title":"J Clin Immunol"},{"key":"10.1053\/j.seminhematol.2021.10.002_bib0025","doi-asserted-by":"crossref","DOI":"10.1016\/j.mayocp.2021.06.006","article-title":"Clinical heterogeneity of the VEXAS syndrome: a case series","author":"Koster","year":"2021","journal-title":"Mayo Clin Proc"},{"issue":"8","key":"10.1053\/j.seminhematol.2021.10.002_bib0026","doi-asserted-by":"crossref","first-page":"1088","DOI":"10.1002\/art.1780330805","article-title":"The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa","volume":"33","author":"Lightfoot","year":"1990","journal-title":"Arthritis Rheum"},{"issue":"6","key":"10.1053\/j.seminhematol.2021.10.002_bib0027","doi-asserted-by":"crossref","first-page":"945","DOI":"10.1309\/AJCPZE77UAPSMDCD","article-title":"The spectrum of histopathologic findings in cutaneous lesions in patients with Still disease","volume":"144","author":"Larson","year":"2015","journal-title":"Am J Clin Pathol"},{"issue":"8","key":"10.1053\/j.seminhematol.2021.10.002_bib0028","first-page":"e10008","article-title":"Adult onset still's disease: a retrospective, single-center study","volume":"12","author":"Hassan","year":"2020","journal-title":"Cureus"},{"issue":"3","key":"10.1053\/j.seminhematol.2021.10.002_bib0029","first-page":"592","article-title":"Severe sore throat as a presenting symptom of adult onset Still's disease: a case series and review of the literature","volume":"24","author":"Nguyen","year":"1997","journal-title":"J Rheumatol"},{"issue":"12","key":"10.1053\/j.seminhematol.2021.10.002_bib0030","doi-asserted-by":"crossref","first-page":"1887","DOI":"10.1200\/JCO.1988.6.12.1887","article-title":"Malignancy-associated Sweet's syndrome: review of the world literature","volume":"6","author":"Cohen","year":"1988","journal-title":"J Clin Oncol"},{"issue":"15","key":"10.1053\/j.seminhematol.2021.10.002_bib0031","doi-asserted-by":"crossref","first-page":"e3033","DOI":"10.1097\/MD.0000000000003033","article-title":"Histiocytoid sweet syndrome is more frequently associated with myelodysplastic syndromes than the classical neutrophilic variant: a comparative series of 62 patients","volume":"95","author":"Ghoufi","year":"2016","journal-title":"Medicine (Baltimore)"},{"issue":"7","key":"10.1053\/j.seminhematol.2021.10.002_bib0032","doi-asserted-by":"crossref","first-page":"741","DOI":"10.1111\/ced.12899","article-title":"Sweet syndrome: long-term follow-up of 138 patients","volume":"41","author":"Marcoval","year":"2016","journal-title":"Clin Exp Dermatol"},{"issue":"2","key":"10.1053\/j.seminhematol.2021.10.002_bib0033","doi-asserted-by":"crossref","first-page":"303-9 e4","DOI":"10.1016\/j.jaad.2017.09.013","article-title":"Sweet syndrome in patients with and without malignancy: a retrospective analysis of 83 patients from a tertiary academic referral center","volume":"78","author":"Nelson","year":"2018","journal-title":"J Am Acad Dermatol"},{"key":"10.1053\/j.seminhematol.2021.10.002_bib0034","doi-asserted-by":"crossref","DOI":"10.1016\/j.jaut.2020.102421","article-title":"Systemic autoinflammatory diseases","volume":"109","author":"Krainer","year":"2020","journal-title":"J Autoimmun"},{"issue":"4","key":"10.1053\/j.seminhematol.2021.10.002_bib0035","doi-asserted-by":"crossref","first-page":"1186","DOI":"10.1002\/art.27340","article-title":"Treatment of polyarteritis nodosa and microscopic polyangiitis without poor-prognosis factors: a prospective randomized study of one hundred twenty-four patients","volume":"62","author":"Ribi","year":"2010","journal-title":"Arthritis Rheum"},{"issue":"1","key":"10.1053\/j.seminhematol.2021.10.002_bib0036","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1002\/art.10922","article-title":"Treatment of polyarteritis nodosa and microscopic polyangiitis with poor prognosis factors: a prospective trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in sixty-five patients","volume":"49","author":"Guillevin","year":"2003","journal-title":"Arthritis Rheum"},{"issue":"Suppl 1","key":"10.1053\/j.seminhematol.2021.10.002_bib0037","doi-asserted-by":"crossref","DOI":"10.1136\/rmdopen-2018-000788","article-title":"Relapsing polychondritis: state of the art on clinical practice guidelines","volume":"4","author":"Rednic","year":"2018","journal-title":"RMD Open"}],"container-title":["Seminars in Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0037196321000482?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0037196321000482?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,22]],"date-time":"2025-09-22T06:27:24Z","timestamp":1758522444000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0037196321000482"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,10]]},"references-count":37,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2021,10]]}},"alternative-id":["S0037196321000482"],"URL":"https:\/\/doi.org\/10.1053\/j.seminhematol.2021.10.002","relation":{},"ISSN":["0037-1963"],"issn-type":[{"value":"0037-1963","type":"print"}],"subject":[],"published":{"date-parts":[[2021,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"VEXAS within the spectrum of rheumatologic disease","name":"articletitle","label":"Article Title"},{"value":"Seminars in Hematology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1053\/j.seminhematol.2021.10.002","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2021 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}