Affiliation:
1. Nuclear Medicine
2. Hematology, AP-HP Avicenne University Hospital, Bobigny, France.
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. 18F-FDG PET/CT showed thoracic aortitis, a rare involvement during VEXAS syndrome. Corticosteroid therapy monitored with 18F-FDG PET/CT led to a complete metabolic response.
Publisher
Ovid Technologies (Wolters Kluwer Health)
Subject
Radiology, Nuclear Medicine and imaging,General Medicine
Cited by
6 articles.
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1. Síndrome de VEXAS: manifestaciones clínicas, diagnóstico y tratamiento;Reumatología Clínica;2024-01
2. VEXAS syndrome: Clinical manifestations, diagnosis, and treatment;Reumatología Clínica (English Edition);2024-01
3. Clinical characteristics, disease trajectories and management of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome: a systematic review;Rheumatology International;2023-12-21
4. Polyarteritis Nodosa: Old Disease, New Etiologies;International Journal of Molecular Sciences;2023-11-23
5. VEXAS syndrome: Clinical, hematologic features and a practical approach to diagnosis and management;American Journal of Hematology;2023-11-11