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American College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel.
Mekinian A, Georgin-Lavialle S, Ferrada MA, Savic S, Koster MJ, Kosmider O, Comont T, Heiblig M, Arostegui JI, Bosco A, Bourguiba R, Calvo KR, Cargo C, Cattaneo C, Chasset F, Coelho H, Campochiaro C, Crisafulli F, Ducharme-Benard S, Faria R, Franceschini F, Frassi M, Groarke EM, Gurnari C, Hakobyan Y, Jamilloux Y, Jurcut C, Kirino Y, Kulasekararaj A, Kunimoto H, Madigan LM, Mann HF, Marvisi C, Milchert M, Morais S, Sockel K, Muratore F, Nakajima H, Patnaik MM, Regadas L, Robin M, Rutgers A, Salvarani C, Sammel AM, Seebach J, Sujobert P, Tomelleri A, Urbanski G, Vandergheynst F, Vieira R, Viswanatha DS, Więsik-Szewczyk E, Diral E, Terrier B, Patel BA, Fenaux P, Grayson PC, Beck DB; International VEXAS working group, and with endorsement of EuroBloodNet, the European Reference Network in Rare Hematological Diseases. Mekinian A, et al. Arthritis Rheumatol. 2026 Mar;78(3):509-522. doi: 10.1002/art.43287. Epub 2025 Aug 11. Arthritis Rheumatol. 2026. PMID: 40787890 Free PMC article.
Guidelines for the genetic diagnosis of hereditary recurrent fevers.
Shinar Y, Obici L, Aksentijevich I, Bennetts B, Austrup F, Ceccherini I, Costa JM, De Leener A, Gattorno M, Kania U, Kone-Paut I, Lezer S, Livneh A, Moix I, Nishikomori R, Ozen S, Phylactou L, Risom L, Rowczenio D, Sarkisian T, van Gijn ME, Witsch-Baumgartner M, Morris M, Hoffman HM, Touitou I; European Molecular Genetics Quality Network. Shinar Y, et al. Ann Rheum Dis. 2012 Oct;71(10):1599-605. doi: 10.1136/annrheumdis-2011-201271. Epub 2012 Jun 1. Ann Rheum Dis. 2012. PMID: 22661645 Free PMC article.
The 2021 EULAR/American College of Rheumatology points to consider for diagnosis, management and monitoring of the interleukin-1 mediated autoinflammatory diseases: cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic syndrome, mevalonate kinase deficiency, and deficiency of the interleukin-1 receptor antagonist.
Romano M, Arici ZS, Piskin D, Alehashemi S, Aletaha D, Barron KS, Benseler S, Berard R, Broderick L, Dedeoglu F, Diebold M, Durrant KL, Ferguson P, Foell D, Hausmann J, Jones OY, Kastner DL, Lachmann HJ, Laxer RM, Rivera D, Ruperto N, Simon A, Twilt M, Frenkel J, Hoffman H, de Jesus AA, Kuemmerle-Deschner JB, Ozen S, Gattorno M, Goldbach-Mansky R, Demirkaya E. Romano M, et al. Ann Rheum Dis. 2022 Jul;81(7):907-921. doi: 10.1136/annrheumdis-2021-221801. Epub 2022 May 27. Ann Rheum Dis. 2022. PMID: 35623638 Free article.
Consensus protocols for the diagnosis and management of the hereditary autoinflammatory syndromes CAPS, TRAPS and MKD/HIDS: a German PRO-KIND initiative.
Hansmann S, Lainka E, Horneff G, Holzinger D, Rieber N, Jansson AF, Rösen-Wolff A, Erbis G, Prelog M, Brunner J, Benseler SM, Kuemmerle-Deschner JB. Hansmann S, et al. Pediatr Rheumatol Online J. 2020 Feb 17;18(1):17. doi: 10.1186/s12969-020-0409-3. Pediatr Rheumatol Online J. 2020. PMID: 32066461 Free PMC article.