2025 ACR指南声明:VEXAS综合征的诊断和管理

发布日期:
2025-08-11
英文标题:
American College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel
出处:
Arthritis Rheumatol . 2025 Aug 11.
摘要:
VEXAS(Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic)综合征是罕见的晚发性自身炎症性疾病,其核心特征是由体细胞突变引起的泛素激活酶E1(UBA1基因)功能异常。该病临床表现复杂多样,涉及多系统炎症和血液学异常,且具有较高的致残率和死亡率。本文主要针对哪些患者需要检测、如何检测VEXAS、在VEXAS的背景下如何处理MDS以及管理方面的考虑提供专家指导。 展开

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littlehema
于2025-08-12上传
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美国风湿病学会

The American College of Rheumatology's mission is advancing rheumatology. The organization is for physicians, health professionals, and scientists that meets the mission through programs of education, research, advocacy and practice support. The ACR provides professional education for its members through several venues. The Annual Scientific Meeting, held each fall, is the premier scientific meeting devoted to the rheumatic diseases. This meeting draws thousands of rheumatologists and arthritis health professionals from around the world. A winter rheumatology symposium, spring clinical meetings, and other topical conferences round out the ACR's educational offerings.

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