{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T04:52:33Z","timestamp":1792212753679,"version":"4.4.0"},"reference-count":15,"publisher":"Springer Science and Business Media LLC","issue":"11","license":[{"start":{"date-parts":[[2022,9,14]],"date-time":"2022-09-14T00:00:00Z","timestamp":1663113600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2022,9,14]],"date-time":"2022-09-14T00:00:00Z","timestamp":1663113600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Leukemia"],"published-print":{"date-parts":[[2022,11]]},"DOI":"10.1038\/s41375-022-01698-8","type":"journal-article","created":{"date-parts":[[2022,9,14]],"date-time":"2022-09-14T14:02:58Z","timestamp":1663164178000},"page":"2739-2742","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":97,"title":["A Phase II prospective trial of azacitidine in steroid-dependent or refractory systemic autoimmune\/inflammatory disorders and VEXAS syndrome associated with MDS and CMML"],"prefix":"10.1038","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2849-3049","authenticated-orcid":false,"given":"Arsene","family":"Mekinian","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5035-4470","authenticated-orcid":false,"given":"Lin Pierre","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sylvie","family":"Chevret","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristell","family":"Desseaux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurent","family":"Pascal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thibaut","family":"Comont","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandre","family":"Maria","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"Peterlin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louis","family":"Terriou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maud","family":"D\u2019Aveni Piney","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0143-5327","authenticated-orcid":false,"given":"Marie-Pierre","family":"Gourin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7027-040X","authenticated-orcid":false,"given":"Norbert","family":"Vey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Odile Beyne","family":"Rauzy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Grobost","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Holy","family":"Bezanahary","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Dimicoli-Salazar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"Banos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Wickenhauser","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benoit","family":"De Renzis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3463-0089","authenticated-orcid":false,"given":"Eric","family":"Durot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shanti","family":"Natarajan-Am\u00e9","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurent","family":"Voillat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fatiha","family":"Chermat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karine","family":"Lemaire","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Jachiet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chantal","family":"Himberlin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sylvain","family":"Th\u00e9pot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jose Miguel Torregrosa","family":"Diaz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurent","family":"Frenzel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7651-9189","authenticated-orcid":false,"given":"Emmanuel","family":"Gyan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0434-265X","authenticated-orcid":false,"given":"Guillaume","family":"Denis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"Hirsch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6021-4057","authenticated-orcid":false,"given":"Olivier","family":"Kosmider","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9020-8766","authenticated-orcid":false,"given":"Lionel","family":"Ades","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Fain","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"Fenaux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,9,14]]},"reference":[{"key":"1698_CR1","doi-asserted-by":"publisher","first-page":"291","DOI":"10.1093\/rheumatology\/kev294","volume":"55","author":"A Mekinian","year":"2016","unstructured":"Mekinian A, Grignano E, Braun T, Decaux O, Liozon E, Costedoat-Chalumeau N, et al. Systemic inflammatory and autoimmune manifestations associated with myelodysplastic syndromes and chronic myelomonocytic leukaemia: a French multicentre retrospective study. Rheumatology. 2016;55:291\u2013300.","journal-title":"Rheumatology"},{"key":"1698_CR2","doi-asserted-by":"publisher","first-page":"2720","DOI":"10.1038\/s41375-021-01152-1","volume":"35","author":"L-P Zhao","year":"2021","unstructured":"Zhao L-P, Boy M, Azoulay C, Clappier E, S\u00e9bert M, Amable L, et al. Genomic landscape of MDS\/CMML associated with systemic inflammatory and autoimmune disease. Leukemia. 2021;35:2720\u20134.","journal-title":"Leukemia"},{"key":"1698_CR3","doi-asserted-by":"publisher","first-page":"2628","DOI":"10.1056\/NEJMoa2026834","volume":"383","author":"DB Beck","year":"2020","unstructured":"Beck DB, Ferrada MA, Sikora KA, Ombrello AK, Collins JC, Pei W, et al. Somatic mutations in UBA1 and severe adult-onset autoinflammatory disease. N Engl J Med. 2020;383:2628\u201338.","journal-title":"N Engl J Med."},{"key":"1698_CR4","doi-asserted-by":"publisher","first-page":"2731","DOI":"10.1038\/s41375-021-01353-8","volume":"35","author":"L-P Zhao","year":"2021","unstructured":"Zhao L-P, Schell B, S\u00e9bert M, Kim R, Lemaire P, Boy M, et al. Prevalence of UBA1 mutations in MDS\/CMML patients with systemic inflammatory and auto-immune disease. Leukemia. 2021;35:2731\u20133.","journal-title":"Leukemia"},{"key":"1698_CR5","first-page":"3591","volume":"137","author":"PC Grayson","year":"2021","unstructured":"Grayson PC, Patel BA, Young NS. VEXAS syndrome. Blood. 2021;137:3591\u20134.","journal-title":"Blood"},{"key":"1698_CR6","doi-asserted-by":"publisher","first-page":"1016","DOI":"10.1111\/bjh.16206","volume":"189","author":"P Fenaux","year":"2020","unstructured":"Fenaux P, Platzbecker U, Ades L. How we manage adults with myelodysplastic syndrome. Br J Haematol. 2020;189:1016\u201327.","journal-title":"Br J Haematol"},{"key":"1698_CR7","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1016\/j.leukres.2016.02.005","volume":"43","author":"J-B Fraison","year":"2016","unstructured":"Fraison J-B, Mekinian A, Grignano E, Kahn J-E, Arlet J-B, Decaux O, et al. Efficacy of Azacitidine in autoimmune and inflammatory disorders associated with myelodysplastic syndromes and chronic myelomonocytic leukemia. Leuk Res. 2016;43:13\u201317.","journal-title":"Leuk Res"},{"key":"1698_CR8","doi-asserted-by":"publisher","first-page":"419","DOI":"10.1182\/blood-2005-10-4149","volume":"108","author":"BD Cheson","year":"2006","unstructured":"Cheson BD, Greenberg PL, Bennett JM, Lowenberg B, Wijermans PW, Nimer SD, et al. Clinical application and proposal for modification of the International Working Group (IWG) response criteria in myelodysplasia. Blood. 2006;108:419\u201325.","journal-title":"Blood"},{"key":"1698_CR9","doi-asserted-by":"publisher","first-page":"130","DOI":"10.1002\/ajh.23368","volume":"88","author":"D Merkel","year":"2013","unstructured":"Merkel D, Filanovsky K, Gafter-Gvili A, Vidal L, Aviv A, Gatt ME, et al. Predicting infections in high-risk patients with myelodysplastic syndrome\/acute myeloid leukemia treated with azacitidine: a retrospective multicenter study. Am J Hematol. 2013;88:130\u20134.","journal-title":"Am J Hematol"},{"key":"1698_CR10","doi-asserted-by":"publisher","first-page":"969","DOI":"10.1111\/bjh.17893","volume":"196","author":"T Comont","year":"2022","unstructured":"Comont T, Heiblig M, Rivi\u00e8re E, Terriou L, Rossignol J, Bouscary D, et al. Azacitidine for patients with Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome (VEXAS) and myelodysplastic syndrome: data from the French VEXAS registry. Br J Haematol. 2022;196:969\u201374.","journal-title":"Br J Haematol"},{"key":"1698_CR11","doi-asserted-by":"publisher","first-page":"e661","DOI":"10.1097\/HS9.0000000000000661","volume":"5","author":"MHGP Raaijmakers","year":"2021","unstructured":"Raaijmakers MHGP, Hermans M, Aalbers A, Rijken M, Dalm VASH, van Daele P, et al. Azacytidine treatment for VEXAS syndrome. Hemasphere. 2021;5:e661.","journal-title":"Hemasphere"},{"key":"1698_CR12","doi-asserted-by":"publisher","first-page":"3499","DOI":"10.1093\/rheumatology\/keaa282","volume":"59","author":"L Ricard","year":"2020","unstructured":"Ricard L, Hirsch P, Largeaud L, Deswarte C, Jachiet V, Mohty M, et al. Clonal haematopoiesis is increased in early onset in systemic sclerosis. Rheumatology. 2020;59:3499\u2013504.","journal-title":"Rheumatology."},{"key":"1698_CR13","doi-asserted-by":"publisher","first-page":"1727","DOI":"10.1182\/blood.2020010163","volume":"138","author":"JS Hecker","year":"2021","unstructured":"Hecker JS, Hartmann L, Rivi\u00e8re J, Buck MC, van der Garde M, Rothenberg-Thurley M, et al. CHIP & HIPs: clonal hematopoiesis is common in hip arthroplasty patients and associates with autoimmune disease. Blood. 2021;138:1727\u201332.","journal-title":"Blood"},{"key":"1698_CR14","doi-asserted-by":"publisher","first-page":"1147","DOI":"10.1038\/leu.2011.71","volume":"25","author":"R Itzykson","year":"2011","unstructured":"Itzykson R, Kosmider O, Cluzeau T, Mansat-De Mas V, Dreyfus F, Beyne-Rauzy O, et al. Impact of TET2 mutations on response rate to azacitidine in myelodysplastic syndromes and low blast count acute myeloid leukemias. Leukemia. 2011;25:1147\u201352.","journal-title":"Leukemia"},{"key":"1698_CR15","doi-asserted-by":"publisher","first-page":"1196","DOI":"10.3324\/haematol.2012.074823","volume":"98","author":"B Costantini","year":"2013","unstructured":"Costantini B, Kordasti SY, Kulasekararaj AG, Jiang J, Seidl T, Abellan PP, et al. The effects of 5-azacytidine on the function and number of regulatory T cells and T-effectors in myelodysplastic syndrome. Haematologica. 2013;98:1196\u2013205.","journal-title":"Haematologica"}],"container-title":["Leukemia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.nature.com\/articles\/s41375-022-01698-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41375-022-01698-8","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41375-022-01698-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,10,27]],"date-time":"2022-10-27T14:12:22Z","timestamp":1666879942000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/s41375-022-01698-8"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,9,14]]},"references-count":15,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2022,11]]}},"alternative-id":["1698"],"URL":"https:\/\/doi.org\/10.1038\/s41375-022-01698-8","relation":{},"ISSN":["0887-6924","1476-5551"],"issn-type":[{"value":"0887-6924","type":"print"},{"value":"1476-5551","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,9,14]]},"assertion":[{"value":"28 June 2022","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 August 2022","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 September 2022","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"14 September 2022","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"A Mekinian is an investigator of Celgene, Roche, Chugai founded trials with APHP and H\u00f4pital 15\u201320 promotion; and received several fees for advisory boards from LFB, Sanofi, Shire, and Celgene. The other authors declare no competing interests.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}]}}