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Aortitis in VEXAS Syndrome.
Intern Med
March 2025
Department of Internal Medicine, Tama Nambu Chiiki Hospital, Japan.
Aortitis PET Imaging in VEXAS Syndrome: A Case Report.
Clin Nucl Med
February 2023
From the Departments of Nuclear Medicine.
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.
View Article and Find Full Text PDFA case of VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) with decreased oxidative stress levels after oral prednisone and tocilizumab treatment.
Front Med (Lausanne)
December 2022
Department of Dermatology, Gifu University Graduate School of Medicine, Gifu, Japan.
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome has recently been described as an autoinflammatory disease associated with severe adult-onset inflammatory manifestations. The various clinical manifestations include recurrent high-grade fever, neutrophilic dermatoses, cutaneous vasculitis, chondritis of the ear and nose, pulmonary infiltrates, cytopenia, uveitis, gastrointestinal pain or inflammation, aortitis, hepatosplenomegaly, and hematological disorders. VEXAS syndrome is caused by somatic mutations of the ubiquitin-like modifier activating enzyme 1 () gene in myeloid-lineage cells.
View Article and Find Full Text PDFSpectrum of Large and Medium Vessel Vasculitis in Adults: Primary Vasculitides, Arthritides, Connective Tissue, and Fibroinflammatory Diseases.
Curr Rheumatol Rep
November 2022
Department of Rheumatology and Immunology, Inselspital, University Hospital, University of Bern, Freiburgstrasse, CH-3010, Bern, Switzerland.
Purpose Of Review: To provide a comprehensive overview of the spectrum of large and medium vessel vasculitis in adults with primary vasculitides, arthritides, connective tissue, and fibroinflammatory diseases as well as vasculitis mimics, for an efficient differential diagnosis and initial diagnostic approach.
Recent Findings: Imaging has had a tremendous impact on the diagnosis of medium to large vessel vasculitis, now often replacing histopathologic confirmation and identifying new disease manifestations (e.g.
Mimickers of Large Vessel Giant Cell Arteritis.
J Clin Med
January 2022
INSERM, EFS BFC, UMR 1098, RIGHT Graft-Host-Tumor Interactions/Cellular and Genetic Engineering, Bourgogne Franche-Comté University, 21000 Dijon, France.
Giant cell arteritis (GCA) is a large-vessel granulomatous vasculitis occurring in patients over 50-year-old. Diagnosis can be challenging because there is no specific biological test or other diagnoses to consider. Two main phenotypes of GCA are distinguished and can be associated.
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