S Georgin-Lavialle, B Terrier, A F Guedon, M Heiblig, T Comont, E Lazaro, V Lacombe, L Terriou, S Ardois, J-D Bouaziz, A Mathian, G Le Guenno, A Aouba, R Outh, A Meyer, M Roux-Sauvat, M Ebbo, L P Zhao, A Bigot, Y Jamilloux, V Guillotin, E Flamarion, P Henneton, G Vial, V Jachiet, J Rossignol, S Vinzio, T Weitten, J Vinit, C Deligny, S Humbert, M Samson, N Magy-Bertrand, T Moulinet, R Bourguiba, T Hanslik, C Bachmeyer, M Sebert, M Kostine, B Bienvenu, P Biscay, E Liozon, L Sailler, F Chasset, A Audemard-Verger, E Duroyon, G Sarrabay, F Borlot, C Dieval, T Cluzeau, P Marianetti, H Lobbes, G Boursier, M Gerfaud-Valentin, J Jeannel, A Servettaz, S Audia, M Larue, B Henriot, B Faucher, J Graveleau, B de Sainte Marie, J Galland, L Bouillet, C Arnaud, L Ades, F Carrat, P Hirsch, P Fenaux, O Fain, P Sujobert, O Kosmider, A Mekinian
BACKGROUND: A new autoinflammatory syndrome related to somatic mutations of UBA1 was recently described and called VEXAS syndrome ('Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome'). OBJECTIVES: To describe clinical characteristics, laboratory findings and outcomes of VEXAS syndrome. METHODS: One hundred and sixteen patients with VEXAS syndrome were referred to a French multicentre registry between November 2020 and May 2021...
March 2022: British Journal of Dermatology