Monogenic autoinflammatory syndromes with features of systemic vasculitis: a new field of rheumatology

T. Beketova, M. Beketova, E. Nasonov
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Abstract

The article is dedicated to a new actual problem in rheumatology: vasculitis and vasculitis-like manifestations in monogenic autoinflammatory syndromes in adult. The features of the clinical course of the rarely diagnosed VEXAS syndrome, as well as the SAVI and COPA syndromes, which sometimes occur in adults, are considered. Promising directions of future treatment are discussed.
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具有系统性血管炎特征的单基因自身炎症综合征:风湿病学的一个新领域
本文致力于风湿病学中的一个新的实际问题:成人单基因自身炎症综合征中的血管炎和血管炎样表现。考虑到罕见诊断的VEXAS综合征的临床病程特点,以及有时发生在成人中的SAVI和COPA综合征。讨论了今后的治疗方向。
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