Aortitis PET Imaging in VEXAS Syndrome: A Case Report. (7th February 2023)

• Record Type: Journal Article
• Authors: Grambow-Velilla, Julia; Braun, Thorsten; Pop, Gabriel; Louzoun, Astrid; Soussan, Michael
• Is Part Of: Clinical nuclear medicine. Volume 48:Number 2(2023)
• Page Start: e67
• Page End: e68
• Publication Date: 2023-02-07
• Subjects: 18F-FDG PET/CT -- aortitis -- large-vessel vasculitis (LVV) -- VEXAS syndrome; Nuclear medicine -- Periodicals; Radioisotope scanning -- Periodicals; Nuclear Medicine -- Periodicals; 616.07575
• DOI: 10.1097/RLU.0000000000004506
• Languages: English
• ISSNs: 0363-9762

Abstract

Abstract: VEXAS (vacuoles, E1 enzyme, X-LINKED, autoinflammatory, somatic) syndrome is a complex inflammatory disease associated with somatic mutations of the ubiquitin-like modifier activating enzyme 1 ( UBA1 ) gene. A 75-year-old man with a medical history of thrombophlebitis, leukocytoclastic vasculitis, chronic inflammatory arthralgia, elevated inflammatory markers, and anemia was diagnosed with VEXAS syndrome. 18 F-FDG PET/CT showed thoracic aortitis, a rare involvement during VEXAS syndrome. Corticosteroid therapy monitored with 18 F-FDG PET/CT led to a complete metabolic response.