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Inflammatory Signatures in VEXAS Syndrome, Myelodysplasia Cutis, and Sweet Syndrome.
JAMA Dermatol
March 2025
Service de Dermatologie et Allergologie, Faculté de Médecine, Sorbonne Université, Hôpital Tenon, Assistance Publique-Hôpitaux de Paris, Paris, France.
Importance: VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a monogenic disease caused by UBA1 somatic variants in hematopoietic progenitor cells, mostly involving adult men. It is associated with inflammatory-related symptoms, frequently involving the skin and hematological disorders. Recently described myelodysplasia cutis (MDS-cutis) is a cutaneous manifestation of myelodysplasia in which clonal myelodysplastic cells infiltrate the skin.
View Article and Find Full Text PDFVEXAS Syndrome: A Perspective Focus on Genetics and Hematological Manifestations.
Genet Test Mol Biomarkers
March 2025
Department of Clinical Sciences, Pathology, University of Central Florida College of Medicine, Orlando, Florida, USA.
Aortitis in VEXAS Syndrome.
Intern Med
March 2025
Department of Internal Medicine, Tama Nambu Chiiki Hospital, Japan.
Efficacy and safety profile of biotechnological agents and Janus kinase inhibitors in VEXAS syndrome: data from the international AIDA Network VEXAS registry.
Front Pharmacol
February 2025
Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.
Background: VEXAS syndrome, a recently identified systemic autoinflammatory disorder, poses new diagnostic and management challenges. Based on experience with other autoinflammatory diseases, anti-interleukin (IL)-1, anti-IL-6, anti-tumor necrosis factor (TNF) biotechnological agents, and Janus kinase inhibitors (JAKis) have been widely employed in VEXAS patients. The aim of this study is to evaluate the global effectiveness and safety of biotechnological agents and JAKis using data from the real-world context.
View Article and Find Full Text PDFVacuoles, E1 Enzyme, X-linked, Autoinflammatory, and Somatic (VEXAS) Syndrome: A Case Report.
Cureus
February 2025
Internal Medicine, Baptist Memorial Hospital, Oxford, USA.
VEXAS is an acronym that stands for the technical terms of key descriptors of the condition: vacuoles, E1 enzyme, X-linked, autoinflammatory, and somatic (VEXAS) syndrome, which is a recently identified autoinflammatory disorder primarily affecting men older than 50 years of age. It is commonly associated with a somatic mutation in the X-linked ubiquitin-activating enzyme-encoding gene. This condition manifests in a range of hematologic and systemic inflammatory symptoms, such as cytopenias, recurrent fevers, and an elevated risk for hematologic malignancies like myelodysplastic syndrome (MDS).
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