SynthesisRheumatology international2024
Clinical characteristics, disease trajectories and management of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome: a systematic review.
Synthesis in Rheumatology international, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers, 1 of them a synthesis that pooled it.
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Who cites it
14 citing papers in PubMed, 1 synthesis or guideline pooled it, 16 citations in OpenAlex.
- Emerging treatment approaches for VEXAS syndrome: a systematic review and meta-analysis.Annals of hematology · 2025Pooled it
- Diagnostic and Therapeutic Challenge: VEXAS Syndrome with Multisystem Inflammation and Myelodysplasia: A Case Report.Mediterranean journal of rheumatology · 2026Article
- VEXAS syndrome unmasked from relapsing polychondritis and infection mimicry: a case-based review.Rheumatology international · 2026Review
- VEXAS syndrome with atypical presentation: A case report and review of eosinophils in cutaneous biopsies.JAAD case reports · 2026Article
- Exploring glucocorticoid dose-response patterns in VEXAS syndrome: a pilot retrospective study.Rheumatology international · 2026Review
- Renal Manifestations in VEXAS Syndrome: A Systematic Review of Clinical Features, Pathology, and Outcomes.Mediterranean journal of rheumatology · 2026Article
- Case Report: Early-onset VEXAS syndrome with recurrent pulmonary inflammation and myelodysplasia: a diagnostic and therapeutic challenge.Frontiers in immunology · 2026Article
- Recurrent fever and association with severe organ involvement, mortality and treatment outcomes in VEXAS syndrome: data from the AIDA Network.Frontiers in immunology · 2026Article
- Coexistence of VEXAS Syndrome and Chronic Hepatitis B Virus Infection: a Case Report and Literature Review.Clinical reviews in allergy & immunology · 2025Review
- From diagnostic uncertainty to targeted therapy: a case-based review of VEXAS syndrome.Rheumatology international · 2025Review
- Refractory fever, pulmonary infiltrates, and auricular chondritis: a rare case of VEXAS syndrome following COVID-19 infection in the emergency department.World journal of emergency medicine · 2025Article
- VEXAS Syndrome: An Overlooked Threat.Balkan medical journal · 2025Article
- Altered IgG4 serum levels in VEXAS syndrome - a retrospective monocentric cohort study.Rheumatology international · 2025Article
- Promise of Jak Inhibition in the Management of VEXAS, Case Report with Review of the Literature.Open access rheumatology : research and reviews · 2025Article
Corrections and comments
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Authors and funding
5 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundVEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly discovered autoinflammatory condition characterised by somatic mutation of the UBA1 gene. The syndrome leads to multi-system inflammation affecting predominantly the skin, lungs and bone marrow.
methodsWe undertook a systematic review of the multisystem features and genotypes observed in VEXAS syndrome. Articles discussing VEXAS syndrome were included. Medline, Embase and Cochrane databases were searched. Information was extracted on: demographics, type and prevalence of clinical manifestations, genetic mutations and treatment. Meta-analysis using a random effects model was used to determine pooled estimates of serum markers.
resultsFrom 303 articles, 90 were included, comprising 394 patients with VEXAS. 99.2% were male, with a mean age of 67.1 years (SD 8.5) at disease onset. The most frequent diagnoses made prior to VEXAS were: relapsing polychondritis (n = 59); Sweet's syndrome (n = 24); polyarteritis nodosa (n = 11); and myelodysplastic syndrome (n = 10). Fever was reported in 270 cases (68.5%) and weight loss in 79 (20.1%). Most patients had haematological (n = 342; 86.8%), dermatological (n = 321; 81.5%), pulmonary (n = 297; 75.4%%) and musculoskeletal (n = 172; 43.7%) involvement, although other organ manifestations of varying prevalence were also recorded. The most commonly reported mutations were "c.122T > C pMET41Thr" (n = 124), "c.121A > G pMET41Val" (n = 62) and "c.121A > C pMet41Leu" (n = 52). Most patients received glucocorticoids (n = 240; 60.9%) followed by methotrexate (n = 82; 20.8%) and IL-6 inhibitors (n = 61, 15.4%). One patient underwent splenectomy; 24 received bone marrow transplants.
conclusionVEXAS syndrome is a rare disorder affecting predominantly middle-aged men. This is the first systematic review to capture clinical manifestations, genetics and treatment of reported cases. Further studies are needed to optimise treatment and subsequently reduce morbidity and mortality.
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