Service d'Hématologie Clinique, Hôpital Lyon Sud, Hospices Civils de Lyon, Pierre Bénite, France.
National Institute of Arthritis, Musculoskeletal, and Skin Diseases, National Institutes of Health, Bethesda, MD.
Blood. 2022 Aug 25;140(8):927-931. doi: 10.1182/blood.2022016642.
VEXAS syndrome (vacuoles in myeloid progenitors, E1 ubiquitin activating enzyme, X-linked, autoinflammatory manifestations and somatic) is an autoinflammatory condition caused by somatically acquired 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.
VEXAS 综合征（髓系前体细胞空泡、E1 泛素激活酶、X 连锁、自身炎症表现和体细胞）是一种由体细胞获得性突变引起的自身炎症性疾病。Heiblig 等人报告了一项针对 30 例 VEXAS 综合征患者的国际回顾性分析，这些患者接受了不同的 Janus 激酶（JAK）抑制剂治疗，结果发现 JAK1/2 抑制剂芦可替尼的使用具有令人鼓舞的证据支持，大多数患者的临床缓解和类固醇使用减少。
