Article
American College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel.
Arthritis & rheumatology (Hoboken, N.J.) - 1 Mar 2026
Mekinian Arsene, Georgin-Lavialle Sophie, Ferrada Marcela A, Savic Sinisa, Koster Matthew J, Kosmider Olivier, Comont Thibault, Heiblig Mael, Arostegui Juan I, Bosco Annmarie, Bourguiba Rim, Calvo Katherine R, Cargo Catherine, Cattaneo Chiara, Chasset François, Coelho Henrique, Campochiaro Corrado, Crisafulli Francesca, Ducharme-Benard Stephanie, Faria Raquel, Franceschini Franco, Frassi Micol, Groarke Emma M, Gurnari Carmelo, Hakobyan Yervand, Jamilloux Yvan, Jurcut Ciprian, Kirino Yohei, Kulasekararaj Austin, Kunimoto Hiroyoshi, Madigan Lauren M, Mann Heřman F, Marvisi Chiara, Milchert Marcin, Morais Sara, Sockel Katja, Muratore Francesco, Nakajima Hideaki, Patnaik Mrinal M, Regadas Luísa, Robin Marie, Rutgers Abraham, Salvarani Carlo, Sammel Anthony M, Seebach Joerg, Sujobert Pierre, Tomelleri Alessandro, Urbanski Geoffrey, Vandergheynst Frédéric, Vieira Romana, Viswanatha David S, Więsik-Szewczyk Ewa, Diral Elisa, Terrier Benjamin, Patel Bhavisha A, Fenaux Pierre, Grayson Peter C, Beck David B
Abstract excerpt
OBJECTIVE: Vacuoles E1 enzyme X-linked autoinflammatory somatic syndrome (VEXAS) is a recently identified rare genetic disorder associated with somatic mutations in the UBA1 gene. VEXAS presents with a combination of inflammatory and hematologic manifestations, leading to increased morbidity and mortality. METHODS: Given the variability in disease presentation and the limited number of studies to date, no...
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