ArticleJournal of thrombosis and thrombolysis2022
Thrombosis in VEXAS syndrome.
Article in Journal of thrombosis and thrombolysis, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers, 3 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
22 citing papers in PubMed, 3 syntheses or guidelines pooled it, 51 citations in OpenAlex.
- Clinical features in VEXAS syndrome: a systematic review.Rheumatology (Oxford, England) · 2025Pooled it
- Kidney involvement in VEXAS syndrome: insights from a rare case of secondary amyloidosis and systematic review of renal biopsy-confirmed reports.Clinical rheumatology · 2025Pooled it
- Pulmonary manifestations in VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome: a systematic review.Rheumatology international · 2023Pooled it
- An Italian cartography of VEXAS-related thrombosis.HemaSphere · 2026Article
- VEXAS syndrome unmasked from relapsing polychondritis and infection mimicry: a case-based review.Rheumatology international · 2026Review
- VEXAS Syndrome for the laboratory physician: a case report.Frontiers in immunology · 2026Article
- The First Reported Case of VEXAS Syndrome in Lebanon: Efficacy of Azacitidine as a Therapeutic Option-Case Report.Case reports in hematology · 2026Article
- A cross-sectional survey on VEXAS syndrome: insights from a global expert panel.Clinical rheumatology · 2025Article
- Arterial and venous thrombosis in systemic and monogenic vasculitis.Nature reviews. Rheumatology · 2025Review
- A vexing case of a 73-year-old man with fevers, orbital cellulitis, and asymptomatic interstitial lung disease.Respirology case reports · 2024Article
- [The most frequent febrile syndromes and autoinflammatory diseases in adulthood].Zeitschrift fur Rheumatologie · 2024Review
- Article
- Cutaneous vasculitis in autoinflammatory diseases.The Journal of dermatology · 2024Review
- Review
- VEXAS syndrome as a mimicker of ANCA-associated vasculitis.Rheumatology advances in practice · 2024Article
- VEXAS Syndrome-Diagnostic Clues for the Dermatologist and Gaps in Our Current Understanding: A Narrative Review.JID innovations : skin science from molecules to population health · 2024Review
- VEXAS Syndrome-Review.Global medical genetics · 2023Review
- SARS-CoV-2-induced venous thromboembolism in a patient with VEXAS syndrome.Rheumatology advances in practice · 2023Article
- Systematic search for the UBA1 mutation in men after a first episode of venous thromboembolism: A monocentric study.Journal of thrombosis and haemostasis : JTH · 2022Article
- VEXAS Syndrome: A Novelty in MDS Landscape.Diagnostics (Basel, Switzerland) · 2022Review
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
VEXAS syndrome, an autoinflammatory syndrome due to a Ubiquitin Like Modifier Activating Enzyme 1 (UBA1) somatic mutation, has a high thrombotic burden. We report a case of a 69-year-old male that was diagnosed with VEXAS syndrome who developed venous thromboembolism (VTE). Review of literature of existing VEXAS syndrome cases showed a high thrombotic burden, with the reported incidence of VTE (36.4%) being markedly higher than arterial thrombosis (1.6%), with deep vein thrombosis being more common than pulmonary embolism. Somatic mutation in the UBA1 gene results in decreased ubiquitylation which is a key driver in the development of thrombosis in VEXAS syndrome, due to chronic inflammation and cytokine release from abnormal crosstalk between the intrinsic effector mechanism of innate immune cells, platelets and endothelium resulting in dysregulated haemostasis and endothelial dysfunction. Targeting endothelial dysfunction and reducing inflammatory milieu causing hypercoagulability with immunosuppressants and immunomodulatory agents, together with anticoagulation may be the strategy to prevent recurrent thrombotic events.
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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.