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

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.

Keywords

• syndrome vexas
• syndrome savi
• syndrome copa
• systemic vasculitis
• monogenic autoinflammatory syndrome
• vasculopathy