ArticleBlood2024
Venous and arterial thrombosis in patients with VEXAS syndrome.
Article in Blood, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers, 3 of them syntheses that pooled it.
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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
33 citing papers in PubMed, 3 syntheses or guidelines pooled it.
- American College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel.Arthritis & rheumatology (Hoboken, N.J.) · 2026Guideline
- Vacuolated Marrow Cytopenias from Copper Deficiency toInternational journal of molecular sciences · 2025Pooled it
- Emerging treatment approaches for VEXAS syndrome: a systematic review and meta-analysis.Annals of hematology · 2025Pooled it
- An Italian cartography of VEXAS-related thrombosis.HemaSphere · 2026Article
- SomaticBlood neoplasia · 2026Article
- VEXAS Mimicking Antiphospholipid Syndrome: Diagnostic Challenges of False-Positive Antiphospholipid Antibodies.International journal of molecular sciences · 2026Article
- VEXAS syndrome unmasked from relapsing polychondritis and infection mimicry: a case-based review.Rheumatology international · 2026Review
- Superficial femoral artery thrombosis after PFNA in an intertrochanteric fracture patient with sarcopenia and copd: a rare case report.BMC geriatrics · 2026Article
- Article
- Clonal Hematopoiesis of Indeterminate Potential (CHIP): A Model of Mutation-Driven Thromboinflammation.Cancers · 2026Review
- VEXAS syndrome.Nature reviews. Disease primers · 2026Review
- Reframing-renaming(?)-myelodysplastic syndromes/neoplasms and clonal hematopoiesis of indeterminate potential.Leukemia · 2026Review
- Disease Trajectories and Glucocorticoid Exposure in VEXAS Syndrome Treated with Cytokine-Directed Therapies.Annals of the rheumatic diseases · 2026Article
- VEXAS Syndrome for the laboratory physician: a case report.Frontiers in immunology · 2026Article
- What should neurologists expect to observe in relapsing polychondritis and VEXAS?Frontiers in immunology · 2026Review
- [VEXAS syndrome mimicking relapsing polychondritis: A case report].Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences · 2025Article
- A cross-sectional survey on VEXAS syndrome: insights from a global expert panel.Clinical rheumatology · 2025Article
- [VEXAS syndrome].Zeitschrift fur Rheumatologie · 2025Review
- Exploring patient-reported outcomes and morbidity burden of patients with VEXAS syndrome: a scoping review.EClinicalMedicine · 2025Review
- The pathogenesis, clinical presentations and treatment of monogenic systemic vasculitis.Nature reviews. Rheumatology · 2025Review
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35 authors.
Funding
Abstract
abstractVEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, caused by somatic mutations in UBA1, is an autoinflammatory disorder with diverse systemic manifestations. Thrombosis is a prominent clinical feature of VEXAS syndrome. The risk factors and frequency of thrombosis in VEXAS syndrome are not well described, due to the disease's recent discovery and the paucity of large databases. We evaluated 119 patients with VEXAS syndrome for venous and arterial thrombosis and correlated their presence with clinical outcomes and survival. Thrombosis occurred in 49% of patients, mostly venous thromboembolism (VTE; 41%). Almost two-thirds of VTEs were unprovoked, 41% were recurrent, and 20% occurred despite anticoagulation. The cumulative incidence of VTE was 17% at 1 year from symptom onset and 40% by 5 years. Cardiac and pulmonary inflammatory manifestations were associated with time to VTE. M41L was positively associated specifically with pulmonary embolism by univariate (odds ratio [OR]: 4.58, confidence interval [CI] 1.28-16.21, P = .02) and multivariate (OR: 16.94, CI 1.99-144.3, P = .01) logistic regression. The cumulative incidence of arterial thrombosis was 6% at 1 year and 11% at 5 years. The overall survival of the entire patient cohort at median follow-up time of 4.8 years was 88%, and there was no difference in survival between patients with or without thrombosis (P = .8). Patients with VEXAS syndrome are at high risk of VTE; thromboprophylaxis should administered be in high-risk settings unless strongly contraindicated.
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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.