{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T03:07:54Z","timestamp":1790564874898,"version":"4.1.0"},"reference-count":24,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2023,3,1]],"date-time":"2023-03-01T00:00:00Z","timestamp":1677628800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2023,3,1]],"date-time":"2023-03-01T00:00:00Z","timestamp":1677628800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2024,3,7]],"date-time":"2024-03-07T00:00:00Z","timestamp":1709769600000},"content-version":"am","delay-in-days":372,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","journal.chestnet.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Chest"],"published-print":{"date-parts":[[2023,3]]},"DOI":"10.1016\/j.chest.2022.10.011","type":"journal-article","created":{"date-parts":[[2022,10,19]],"date-time":"2022-10-19T23:32:00Z","timestamp":1666222320000},"page":"575-585","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":63,"title":["Pleuropulmonary Manifestations of Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic (VEXAS) Syndrome"],"prefix":"10.1016","volume":"163","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9906-0024","authenticated-orcid":false,"given":"Raphael","family":"Borie","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie Pierre","family":"Debray","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexis F.","family":"Guedon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arsene","family":"Mekinian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louis","family":"Terriou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentin","family":"Lacombe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estibaliz","family":"Lazaro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aurore","family":"Meyer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexis","family":"Mathian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuel","family":"Ardois","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Vial","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Moulinet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin","family":"Terrier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yvan","family":"Jamilloux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mael","family":"Heiblig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-David","family":"Bouaziz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eve","family":"Zakine","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roderau","family":"Outh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sylvie","family":"Groslerons","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adrien","family":"Bigot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edouard","family":"Flamarion","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Kostine","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierrick","family":"Henneton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastien","family":"Humbert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnaud","family":"Constantin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maxime","family":"Samson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadine Magy","family":"Bertrand","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascal","family":"Biscay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Celine","family":"Dieval","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Herve","family":"Lobbes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juliette","family":"Jeannel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amelie","family":"Servettaz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leo","family":"Adelaide","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie","family":"Graveleau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin","family":"de Sainte-Marie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joris","family":"Galland","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vivien","family":"Guillotin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eug\u00e9nie","family":"Duroyon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Templ\u00e9","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rim","family":"Bourguiba","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Georgin Lavialle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Kosmider","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Audemard-Verger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julien","family":"Haroche","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zahir","family":"Amoura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Micheline","family":"Pha","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miguel","family":"Hie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kilifa","family":"Meghit","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Murielle","family":"Rondeau-Lutz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Christophe","family":"Weber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"27","key":"10.1016\/j.chest.2022.10.011_bib1","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\u00a0Engl J Med"},{"issue":"9","key":"10.1016\/j.chest.2022.10.011_bib2","doi-asserted-by":"crossref","first-page":"1067","DOI":"10.1038\/ncb2804","article-title":"Uba1 functions in Atg7- and Atg3-independent autophagy","volume":"15","author":"Chang","year":"2013","journal-title":"Nat Cell Biol"},{"issue":"1","key":"10.1016\/j.chest.2022.10.011_bib3","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1016\/j.jaci.2021.05.014","article-title":"Adult-onset autoinflammation caused by somatic mutations in UBA1: a Dutch case series of patients with VEXAS","volume":"149","author":"van der Made","year":"2022","journal-title":"J\u00a0Allergy Clin Immunol"},{"issue":"3","key":"10.1016\/j.chest.2022.10.011_bib4","doi-asserted-by":"crossref","first-page":"564","DOI":"10.1111\/bjd.20805","article-title":"Further characterization of clinical and laboratory features occurring in VEXAS syndrome in a large-scale analysis of multicenter case-series of 116 French patients","volume":"186","author":"Georgin-Lavialle","year":"2021","journal-title":"Br J Dermatol"},{"issue":"2","key":"10.1016\/j.chest.2022.10.011_bib5","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1111\/bjh.17679","article-title":"Vacuoles in neutrophil precursors in VEXAS syndrome: diagnostic performances and threshold","volume":"195","author":"Lacombe","year":"2021","journal-title":"Br J Haematol"},{"issue":"4","key":"10.1016\/j.chest.2022.10.011_bib6","doi-asserted-by":"crossref","first-page":"969","DOI":"10.1111\/bjh.17893","article-title":"Azacitidine for patients with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome (VEXAS) and myelodysplastic syndrome: data from the French VEXAS registry","volume":"196","author":"Comont","year":"2021","journal-title":"Br J Haematol"},{"issue":"1","key":"10.1016\/j.chest.2022.10.011_bib7","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"},{"issue":"10","key":"10.1016\/j.chest.2022.10.011_bib8","doi-asserted-by":"crossref","first-page":"e356","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","volume":"60","author":"Sakuma","year":"2021","journal-title":"Rheumatol Oxf Engl"},{"issue":"3","key":"10.1016\/j.chest.2022.10.011_bib9","doi-asserted-by":"crossref","first-page":"697","DOI":"10.1148\/radiol.2462070712","article-title":"Fleischner Society: glossary of terms for thoracic imaging","volume":"246","author":"Hansell","year":"2008","journal-title":"Radiology"},{"issue":"1","key":"10.1016\/j.chest.2022.10.011_bib10","first-page":"1","article-title":"missMDA: a package for handling missing values in multivariate data analysis","volume":"70","author":"Josse","year":"2016","journal-title":"J\u00a0Stat Softw"},{"issue":"10","key":"10.1016\/j.chest.2022.10.011_bib11","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","volume":"73","author":"Ferrada","year":"2021","journal-title":"Arthritis Rheumatol"},{"issue":"22","key":"10.1016\/j.chest.2022.10.011_bib12","doi-asserted-by":"crossref","first-page":"2163","DOI":"10.1056\/NEJMc2102124","article-title":"Mutant UBA1 and severe adult-onset autoinflammatory disease","volume":"384","author":"Arlet","year":"2021","journal-title":"N\u00a0Engl J Med"},{"issue":"3","key":"10.1016\/j.chest.2022.10.011_bib13","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1001\/jamaoto.2021.4092","article-title":"Ear, nose, throat, and bronchial involvements in VEXAS syndrome: specifying the spectrum of clinical features","volume":"148","author":"Beaumesnil","year":"2022","journal-title":"JAMA Otolaryngol Head Neck Surg"},{"issue":"11","key":"10.1016\/j.chest.2022.10.011_bib14","doi-asserted-by":"crossref","first-page":"1349","DOI":"10.1001\/jamadermatol.2021.3344","article-title":"UBA1 variations in neutrophilic dermatosis skin lesions of patients with VEXAS syndrome","volume":"157","author":"Zakine","year":"2021","journal-title":"JAMA Dermatol"},{"issue":"1","key":"10.1016\/j.chest.2022.10.011_bib15","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1186\/s40164-022-00262-5","article-title":"Distinction between clonal and paraclonal cutaneous involvements in VEXAS syndrome","volume":"11","author":"Lacombe","year":"2022","journal-title":"Exp Hematol Oncol"},{"issue":"2","key":"10.1016\/j.chest.2022.10.011_bib16","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1183\/09031936.06.00013505","article-title":"Cryptogenic organising pneumonia","volume":"28","author":"Cordier","year":"2006","journal-title":"Eur Respir J"},{"issue":"11","key":"10.1016\/j.chest.2022.10.011_bib17","doi-asserted-by":"crossref","first-page":"1058","DOI":"10.1056\/NEJMra2116777","article-title":"Cryptogenic organizing pneumonia","volume":"386","author":"King","year":"2022","journal-title":"N\u00a0Engl J Med"},{"issue":"1","key":"10.1016\/j.chest.2022.10.011_bib18","doi-asserted-by":"crossref","first-page":"E361","DOI":"10.1148\/radiol.2021210384","article-title":"Study of thoracic CT in COVID-19: the STOIC Project","volume":"301","author":"Revel","year":"2021","journal-title":"Radiology"},{"issue":"8","key":"10.1016\/j.chest.2022.10.011_bib19","doi-asserted-by":"crossref","first-page":"927","DOI":"10.1182\/blood.2022016642","article-title":"Ruxolitinib is more effective than other JAK inhibitors to treat VEXAS syndrome: a retrospective multi center study","volume":"140","author":"Heiblig","year":"2022","journal-title":"Blood"},{"key":"10.1016\/j.chest.2022.10.011_bib20","article-title":"Looking for somatic mutations in fibrosing interstitial lung diseases","volume":"79","author":"Ba","year":"2021","journal-title":"Respir Med Res"},{"issue":"144","key":"10.1016\/j.chest.2022.10.011_bib21","doi-asserted-by":"crossref","DOI":"10.1183\/16000617.0122-2016","article-title":"Management of suspected monogenic lung fibrosis in a specialised centre","volume":"26","author":"Borie","year":"2017","journal-title":"Eur Respir Rev"},{"issue":"21","key":"10.1016\/j.chest.2022.10.011_bib22","doi-asserted-by":"crossref","first-page":"5607","DOI":"10.1182\/blood-2010-11-322149","article-title":"Syndrome complex of bone marrow failure and pulmonary fibrosis predicts germline defects in telomerase","volume":"117","author":"Parry","year":"2011","journal-title":"Blood"},{"issue":"6","key":"10.1016\/j.chest.2022.10.011_bib23","doi-asserted-by":"crossref","first-page":"1721","DOI":"10.1183\/13993003.02115-2015","article-title":"Prevalence and characteristics of TERT and TERC mutations in suspected genetic pulmonary fibrosis","volume":"48","author":"Borie","year":"2016","journal-title":"Eur Respir J"},{"issue":"10","key":"10.1016\/j.chest.2022.10.011_bib24","doi-asserted-by":"crossref","first-page":"1200","DOI":"10.1164\/rccm.201504-0818OC","article-title":"Idiopathic pulmonary fibrosis in United States automated claims. Incidence, prevalence, and algorithm validation","volume":"192","author":"Esposito","year":"2015","journal-title":"Am J Respir Crit Care Med"}],"container-title":["Chest"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0012369222040004?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0012369222040004?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,15]],"date-time":"2025-09-15T08:49:03Z","timestamp":1757926143000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0012369222040004"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3]]},"references-count":24,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2023,3]]}},"alternative-id":["S0012369222040004"],"URL":"https:\/\/doi.org\/10.1016\/j.chest.2022.10.011","relation":{},"ISSN":["0012-3692"],"issn-type":[{"value":"0012-3692","type":"print"}],"subject":[],"published":{"date-parts":[[2023,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Pleuropulmonary Manifestations of Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic (VEXAS) Syndrome","name":"articletitle","label":"Article Title"},{"value":"Chest","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.chest.2022.10.011","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2022 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}