Results 31 to 40 of about 865 (130)
VEXAS syndrome: a new discovered systemic rheumatic disorder
Rheumatology, 2023 VEXAS syndrome is an adult-onset autoinflammatory disease associated with hematologic symptoms. The disease affects primarily males, and leads to death of a significant proportion of the patients.
Eugeniusz Józef Kucharz
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Frontiers in MedicineBackgroundA substantial overlap in demographic, clinical, and laboratory features can complicate the differential diagnosis between Schnitzler's syndrome and VEXAS syndrome.
Valeria Caggiano +85 more
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ReumatismoVacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a recently characterized disease associated with somatic mutations in the UBA1 gene, which cause dysregulation of ubiquitin-mediated processes.
C. Iannone +7 more
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Case report: VEXAS syndrome and literature review
Frontiers in HematologyVEXAS syndrome (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a novel disorder first described in 2020. Patients are diagnosed by identifying a somatic mutation of the ubiquitin-like modifier-activating enzyme 1 (UBA1) gene.
Can Jones +7 more
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VEXAS syndrome in a female with constitutional monosomy X
EULAR Rheumatology Open: The VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an autoinflammatory disorder that is caused by an acquired deficiency of the UBA1 gene in haematopoietic progenitor cells and predominantly affects elderly males. However,
Nikolas Ruffer +7 more
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Internal Medicine Journal, EarlyView.Abstract Idiopathic multicentric Castleman disease (iMCD) is a rare condition. The pathogenesis is incompletely understood; however, interleukin‐6 (IL‐6) is a major mediator. The clinical presentation is heterogeneous, from mild constitutional symptoms to severe multi‐organ failure.
Dipti Talaulikar +16 more
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Arthritis Care &Research, Volume 78, Issue 6, Page 798-809, June 2026.Objective Relapsing polychondritis (RP) is a rare disease defined by recurrent cartilaginous inflammation. Anti–collagen II (Col2) antibodies have been proposed as a diagnostic biomarker for RP, but their performance characteristics are not well defined.
Karyssa Stonick +6 more
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A Novel Use of Anifrolumab for a Novel Disease
ACR Open Rheumatology, Volume 8, Issue 5, May 2026.Objective To explore the potential role of type‐I interferon (IFN‐I) signaling in autoimmune cytopenias outside the context of systemic lupus erythematosus (SLE), and to evaluate the clinical response to off‐label anifrolumab in two patients with fatigue and immune‐mediated cytopenias who did not meet established criteria for SLE.
Zachary Holtz, Adam Schulz, Alexa Meara
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Clinical Case Reports, Volume 14, Issue 5, May 2026.Graphical abstract illustrating the phenotype‐guided therapeutic approach in severe adult HLH/MAS with SPTCL‐like panniculitis, demonstrating clinical and biochemical response following sequential treatment with therapeutic plasma exchange, intravenous immunoglobulin, and cyclosporine.
Hatem Mousa Taha +2 more
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Navigating through uncertainty—Experience from the UK national VEXAS MDT
British Journal of Haematology, Volume 208, Issue 4, Page 1306-1313, April 2026.Summary The objective of this study was to describe the establishment, structure and influence of the United Kingdom national multidisciplinary team (MDT) for vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic (VEXAS) syndrome and to assess its clinical outputs and perceived value among participating clinicians.
Daniel Pietsch +51 more
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