Ruxolitinib is more effective than other JAK inhibitors to treat VEXAS syndrome: a retrospective multicenter study

作者：Maël Heiblig, Marcela A. Ferrada, Matthew Koster, Thomas Barba, Mathieu Gerfaud‐Valentin, Arsène MEKINIAN, Henrique Guimaraes Barbosa Coelho, Gaëlle Fossard, Fiorenza Barraco, Lionel Galicier, Boris Bienvenu, Pierre Hirsch, Guillaume Vial, Anne Blandine Boutin, J. Galland, G. Le Guenno, Adrien Bigot, Kenneth J. Warrington, Tanaz A. Kermani, Peter C. Grayson, Bhavisha A. Patel, David B. Beck, Yvan Jamilloux, Pierre Fenaux, Pierre Sujobert · 发表于：Blood · 年份：2022 · DOI：10.1182/blood.2022016642 · 被引用次数：215 · 研究领域：Otitis Media and Relapsing Polychondritis、Coagulation, Bradykinin, Polyphosphates, and Angioedema、Autoimmune Bullous Skin Diseases

VEXAS syndrome (vacuoles in myeloid progenitors, E1 ubiquitin activating enzyme, X-linked, autoinflammatory manifestations and somatic) is an autoinflammatory condition caused by somatically acquired UBA1 mutations. Heiblig et al report on an international retrospective analysis of 30 patients with VEXAS syndrome treated with different Janus kinase (JAK) inhibitors, finding encouraging evidence supporting the use of the JAK1/2 inhibitor ruxolitinib with clinical remissions and reductions in steroid use seen in the majority of patients.