ArticleNature communications2024
VEXAS syndrome is characterized by inflammasome activation and monocyte dysregulation.
Article in Nature communications, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 48 papers, 1 of them a synthesis that pooled it.
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Who cites it
48 citing papers in PubMed, 1 synthesis or guideline pooled it, 79 citations in OpenAlex.
- Clinical features in VEXAS syndrome: a systematic review.Rheumatology (Oxford, England) · 2025Pooled it
- Assessing the Frequency of VEXAS-Related Canonical UBA1 Mutations in Myelodysplastic Syndrome Patients.European journal of haematology · 2026Article
- Myelodysplasia in VEXAS syndrome.Blood advances · 2026Review
- What Explains the Pattern of Fibrosis in Chronic Graft-Versus-Host Disease?Clinical transplantation · 2026Review
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- Article
- A case of VEXAS syndrome presenting as macrophage activation syndrome: from chronic inflammation to multi-organ failure.Internal and emergency medicine · 2026Article
- Cell autonomous inflammation in VEXAS is mediated by cGAS-STING.bioRxiv : the preprint server for biology · 2026Article
- Exploring glucocorticoid dose-response patterns in VEXAS syndrome: a pilot retrospective study.Rheumatology international · 2026Review
- Cryptococcal meningitis in a patient with VEXAS syndrome: a case report.BMC rheumatology · 2026Article
- VEXAS syndrome.Nature reviews. Disease primers · 2026Review
- PAXIS: A Randomized, Double-Blind, Placebo-Controlled, Dose-Finding Phase 2 Study (Part 1) Followed by an Open-Label Period (Part 2) to Assess the Efficacy and Safety of Pacritinib in Patients with VEXAS Syndrome.Journal of clinical medicine · 2026Article
- Clinical-Pathological Conference Series from the Medical University of Graz : Case No 183: A 57-year-old farmer with fever, cough, maculopapular rash and bicytopenia.Wiener klinische Wochenschrift · 2026Article
- Comparative Efficacy and Safety of Anakinra and Canakinumab in Patients With VEXAS Syndrome: An International Multicenter Study.Arthritis & rheumatology (Hoboken, N.J.) · 2026Article
- Disease Trajectories and Glucocorticoid Exposure in VEXAS Syndrome Treated with Cytokine-Directed Therapies.Annals of the rheumatic diseases · 2026Article
- A truncatingFrontiers in immunology · 2026Article
- Article
- UBA1 as a proteostasis-innate immunity checkpoint: lessons from vexas syndrome and therapeutic implications.Frontiers in immunology · 2026Review
- Targeting ubiquitination in disease and therapy.Signal transduction and targeted therapy · 2025Review
- [VEXAS syndrome mimicking relapsing polychondritis: A case report].Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences · 2025Article
Corrections and comments
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Authors and funding
34 authors at 13 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Acquired mutations in the UBA1 gene were recently identified in patients with severe adult-onset auto-inflammatory syndrome called VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic). However, the precise physiological and clinical impact of these mutations remains poorly defined. Here we study a unique prospective cohort of VEXAS patients. We show that monocytes from VEXAS are quantitatively and qualitatively impaired and display features of exhaustion with aberrant expression of chemokine receptors. In peripheral blood from VEXAS patients, we identify an increase in circulating levels of many proinflammatory cytokines, including IL-1β and IL-18 which reflect inflammasome activation and markers of myeloid cells dysregulation. Gene expression analysis of whole blood confirms these findings and also reveals a significant enrichment of TNF-α and NFκB signaling pathways that can mediate cell death and inflammation. This study suggests that the control of the nflammasome activation and inflammatory cell death could be therapeutic targets in VEXAS syndrome.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.