VEXAS syndrome in adults: A narrative review of genomic pathogenesis, multisystem phenotypes, diagnostic algorithms, and emerging therapeutic strategies.

VEXAS Syndrome with an Improvement of Chest Shadows Without Immunosuppressive Therapy: A Case Report.

Large-Vessel Vasculitis With Autoimmune Myelodysplastic Syndrome: An Uncommon Case of Large-Vessel Vasculitis With Cytopenia That Is Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic Syndrome (VEXAS) Negative.

Inflammatory cell death and monocyte dysfunction in VEXAS syndrome.

A case of VEXAS syndrome presenting as macrophage activation syndrome: from chronic inflammation to multi-organ failure.

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.

Navigating through uncertainty-Experience from the UK national VEXAS MDT.

In response to Bergonzi et al.: Azacitidine-ruxolitinib combination therapy may permit dose reduction in VEXAS syndrome.

Case Report: Early-onset VEXAS syndrome with recurrent pulmonary inflammation and myelodysplasia: a diagnostic and therapeutic challenge.

Expanding the Clinical Spectrum of VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic) Syndrome: The First Report of Histologically Confirmed Neutrophilic Colitis With Rare Muscular and Pancreatic Involvement.

A vexing fever: Unveiling VEXAS syndrome.

Beyond Sweet Syndrome: Untangling the Threads of Histiocytoid Sweet Syndrome and Cutaneous Myeloid Neoplasia.

Case Report: Diagnostic challenges in VEXAS syndrome with novel ultrastructural lung findings: IgG4-RD and vasculitis as relevant differential diagnoses.

Rheumatology 2050: How our specialty is changing.

A fatal case of VEXAS syndrome with a brief review.

VEXAS syndrome and cancer: Insights about a possible "Tip of the Iceberg". Ambidirectional data from the international AIDA network registries.

Clinical and laboratory markers to distinguish VEXAS from Schnitzler's syndrome: data from the AIDA network registries.

Hemi-Orolingual Angioedema in a Patient With VEXAS Syndrome.

Managing Metastatic Cutaneous Squamous Cell Carcinoma in VEXAS Syndrome: A Therapeutic Challenge.

Rapid cutaneous remission response to tofacitinib and systemic corticosteroids in VEXAS syndrome with a novel UBA1 mutation.

Characteristics and prognostic significance of myelodysplasia-related features in VEXAS syndrome.

Orbital inflammation in VEXAS syndrome.

Hypomethylating agents in vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome (VEXAS): A systematic review.

VEXAS syndrome: a comprehensive review of pathogenesis, clinical spectrum, and therapeutic strategies.

Reversal of third-degree AV block with ruxolitinib in VEXAS-associated myocarditis: a case report.

A Case of VEXAS Syndrome.

Morphologic Deciphering of Hematopoietic Cell Vacuolization: Lessons From VEXAS Syndrome and Other Etiologies.

A rare case of VEXAS syndrome.

VEXAS Syndrome with Vibrio vulnificus Sepsis During Treatment with Tocilizumab: A Case Report.

A Case of VEXAS Syndrome Initially Masked as Myelodysplastic Syndrome: Importance of Marrow Vacuolization and UBA1 Testing: A Case Report.

A Vexing Diagnosis: ENT Presentation of Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) Syndrome.

Polyarteritis nodosa associated with VEXAS syndrome and chronic myelomonocytic leukemia: a case-control study.

VEXAS syndrome with cochleovestibular involvement.

VEXAS syndrome in rheumatology practice: features from a multicenter cohort in north-east Italy.

[VEXAS syndrome mimicking relapsing polychondritis: A case report].

Case Report: VEXAS syndrome with extensive pulmonary, cardiac, and skeletal involvement.

Safety and effectiveness of the combination of 5-azacitidine and ruxolitinib in VEXAS syndrome: A single-centre experience.

Immunogenetics of Behçet's disease: Is there a link to recent phenotypic changes?

Refining the interpretation of drug survival and safety in VEXAS syndrome: the importance of competing risks and exposure time. Comment on the article by Eviatar et al.

Unravelling the cellular mechanisms of VEXAS syndrome.

VEXAS Syndrome Presenting With Pleuritis.

Reinterpreting the efficacy of IL-1 inhibitors in VEXAS syndrome: a matter of superiority or sequencing?: comment on the article by Eviatar et al.

VEXAS syndrome with eosinophilia and pathologically mimicking histiocytosis: a case report.

Establishing a consensus definition of VEXAS flare for clinical research.

Is the combination of Myelodysplastic syndromes and pulmonary fibrosis accidental or inevitable?

Monitoring Variant Allele Fraction in VEXAS Syndrome: A Comparison of Digital PCR and Next-Generation Sequencing.

[What is an undifferentiated systemic autoinflammatory disease in adults? Current state of knowledge in 2025].

Targeting IL-1 in Autoinflammatory Syndromes: Lessons From Canakinumab and VEXAS Syndrome.

Unravelling the Puzzle: Highlighting a Case of VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic) Mimicker Presented With Inflammatory Symptoms and Pancytopenia.

The MAGIC (Mouth and Genital Ulcers With Inflamed Cartilage) of VEXAS: A Case of Overlap Between Behçet Disease and VEXAS Syndrome.

VEXAS Syndrome Presenting as Erythema Nodosum and Pancytopenia: Diagnostic Utility of 18F-FDG PET/CT in Systemic Inflammation.

Vasculitis associated with haematologic malignancies.

VEXAS syndrome presenting with features of Sjögren's disease: a case-based review of VEXAS and autoimmune disease overlap.

Phenotypes of patients with more than one autoinflammatory disease-associated gene variant: overlapping and mixed autoinflammatory disorders.

VEXAS syndrome: cutaneous manifestations and UBA1 gene variants in the diagnosis of a rare autoinflammatory syndrome - Case report.

Independent mechanisms of inflammation and myeloid bias in VEXAS syndrome.

Neutrophils take center stage in VEXAS syndrome pathogenesis.

Decoding VEXAS syndrome: emerging insights into pathogenesis and clinical management.

Joint involvement in VEXAS and non-VEXAS clonal haematopoiesis: two clusters from a multicentre regional cohort.

VEXAS syndrome in a patient with seropositive rheumatoid arthritis: lessons in diagnostic vigilance.

Histiocytoid Sweet Syndrome - A systematic literature review of the clinical and histological characteristics and treatment outcomes.

Interval Changes in Hematopoietic Vacuolization in VEXAS Syndrome with M41T Mutation: Insights into Diagnosis and Disease Progression.

Primary CD34+ cells of patients with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome are highly sensitive to targeted treatment with TAK-243.

Distinct characteristics of VEXAS-causative UBA1 M41 and recurrent functional non-M41 mutations.

High glucocorticoid dependency and limited therapeutic response in Japanese patients with VEXAS syndrome: a multicentre retrospective study.

Respiratory-Predominant Relapsing Polychondritis: The Role of Pet Scan in Making this Challenging Diagnosis.

A Patient with Vexas Syndrome Presenting with Complete Ophthalmoplegia.

Skin Manifestations in VEXAS Syndrome: Specific Clonal Infiltrates versus Non-Specific Reactive Findings.

Periorbital oedema, trismus, myalgia as muscular manifestations of VEXAS syndrome: a case report and narrative literature review.

Coexistence of VEXAS Syndrome and Chronic Hepatitis B Virus Infection: a Case Report and Literature Review.

Diagnostic and Monitoring Strategies for VEXAS Syndrome: Evaluating Sanger Sequencing, NGS, and the SWIM-Score.

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.

Subconjunctival Lesion in VEXAS Syndrome: Expanding the Ocular Disease Spectrum.

Characterizing VEXAS syndrome in women: Findings from an international multicenter study.

JAK Inhibitors for Treatment of VEXAS Syndrome: A Systematic Review of 186 Cases.

Comparative Efficacy and Safety of Anakinra and Canakinumab in Patients With VEXAS Syndrome: An International Multicenter Study.

Clinical Manifestations of VEXAS Syndrome Across a Broad Spectrum of UBA1 Mutation Burden.

Clinicopathological Challenge: Fixed Urticaria-Like Pink Papules in a Patient With Recurrent Fevers and Arthralgias.

Azacitidine as therapy for VEXAS syndrome.

VEXAS syndrome: more than just vacuoles.

[Immuno'logical (The scientific updates you wouldn't dare to read anywhere else): The hidden face of VEXAS syndrome].

Cancer Risk in Autoimmune and Immune-Mediated Diseases: A Narrative Review for Practising Clinicians.

VEXAS Syndrome and Substance Use Disorders: A Large-Scale, Propensity-Matched, Case-Control Analysis Revealing Immune-Mediated Comorbidities.

Diagnostic persistence in geriatric medicine: A case of VEXAS syndrome in an older adult.

A complex case of recurrent bilateral alternating orbital inflammation associated to VEXAS syndrome.

Mapping the infectious burden in VEXAS syndrome: a systematic review and rationale for prevention.

VEXAS syndrome and immune-mediated rheumatic diseases: overlaps in clinical features and mechanisms.

Extensively distributed erythema nodosum in VEXAS syndrome.

UBA1-depleted neutrophils disrupt immune homeostasis and induce VEXAS-like autoinflammatory disease in mice.

From diagnostic uncertainty to targeted therapy: a case-based review of VEXAS syndrome.

[Treatment for VEXAS syndrome with luspatercept combined with cyclosporine A, methylprednisolone, and thalidomide: a case report].

Vacuolated Marrow Cytopenias from Copper Deficiency to UBA1-Mutant VEXAS: Molecular Landscape, Systematic Review, and Cost-Efficient Diagnostic Algorithm.

VEXAS Syndrome: Genetics, Gender Differences, Clinical Insights, Diagnostic Pitfalls, and Emerging Therapies.

Prevalence and outcome of VEXAS syndrome in unrelated hematopoietic cell transplantation for bone marrow failure.

Vacuoles, E1 Enzyme, X-linked, Auto-Inflammatory, Somatic (VEXAS) Syndrome Causing Acute Lower Limb Ischaemia.

Diagnostic performance of four proposed algorithms for suspected VEXAS syndrome.

Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, and Somatic (VEXAS) Syndrome Presenting With Bicytopenia and Vasculitis.

VEXAS: A review of current understandings and emerging treatment strategies.

American College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel.

Bone marrow vacuolization to curative strategies: Evolving paradigms in VEXAS syndrome management.

A cross-sectional survey on VEXAS syndrome: insights from a global expert panel.

Is immunotherapy safe and effective in patients with VEXAS syndrome?

Recent progress in clonal hematopoiesis: expanding the concept.

Impaired cytotoxic function and exhausted phenotype of natural killer cells in VEXAS syndrome.

Promise of Jak Inhibition in the Management of VEXAS, Case Report with Review of the Literature.

Developing efficient predictive models for the diagnosis of VEXAS syndrome.

Refractory fever, pulmonary infiltrates, and auricular chondritis: a rare case of VEXAS syndrome following COVID-19 infection in the emergency department.

Exploring patient-reported outcomes and morbidity burden of patients with VEXAS syndrome: a scoping review.

Fatal haemorrhage in VEXAS syndrome: a lethal complication unveiled.

A Novel Case of Optic Disc Edema Associated With VEXAS Syndrome.

VEXAS syndrome-associated tumefactive demyelination.

Vexas Syndrome in a Moroccan Patient: The Story of a Two-Year Diagnostic Lag.

Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) Syndrome With Multisystem Involvement: Imaging and Genetic Insights From a Case Report.

Characterization of E1 enzyme dependencies in mutant-UBA1 human cells reveals UBA6 as a novel therapeutic target in VEXAS syndrome.

Momelotinib Is Effective in Treatment for VEXAS Syndrome: Two Cases Within the AGMT Austrian Myeloid Registry.

Disease Trajectories and Glucocorticoid Exposure in VEXAS Syndrome Treated with Cytokine-Directed Therapies.

Erythematous Plaques and Systemic Inflammation in VEXAS Syndrome.

Clinical features in VEXAS syndrome: a systematic review.

VEXAS Syndrome and Alzheimer's Disease-Are There Connections?

Infections in VEXAS syndrome: a systematic review of the literature.

Genetic Basis of Autoinflammatory Skin Diseases. Part I. Genetic Pathways of Complex Autoinflammatory Skin Diseases.

[A CASE OF VACUOLES, E1 ENZYME, X-LINKED, AUTOINFLAMMATORY, SOMATIC (VEXAS) SYNDROME PRESENTING WITH DIVERSE PULMONARY LESIONS OVER TIME].

Unravelling VEXAS syndrome: shedding light on a recently recognised medical condition.

Clonal dominance: mutations in VEXAS syndrome take advantage of inflammation.

Efficacy of erythroid-stimulating agent and luspatercept in VEXAS syndrome: A multicenter retrospective study by the FRENVEX group.

Solid organ neoplasms in a patient with VEXAS syndrome.

Unveiling VEXAS Syndrome: When Skin Manifestations and Monoclonal Gammopathy Precede Myeloid-Lineage Hematologic Abnormality.

Kidney involvement in VEXAS syndrome: insights from a rare case of secondary amyloidosis and systematic review of renal biopsy-confirmed reports.

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome: A comprehensive review of cases across different ethnicities.

Bone Marrow Vacuolization at the Crossroads of Specialties: Molecular Insights and Diagnostic Challenges.

A series of cutaneous manifestations in a patient with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome.

Rapid clinical deployment of UBA1 testing in patients with VEXAS syndrome.

VEXAS syndrome caused by a UBA1 mutation is complicated by recurrent infections leading to hemophagocytic lymphohistiocytosis.

Mapping VEXAS-associated and rare UBA1 variants in the United Kingdom: Insights from patient cohorts and the general population.

Treatment outcomes in patients with VEXAS syndrome: a retrospective cohort study.

Treatment outcomes in VEXAS syndrome: response is in the eye of the definer.

Recapitulation of clinical features in a patient-derived xenograft mouse model of VEXAS syndrome.

Clonal hematopoiesis meets an autoinflammatory disease: the new paradigm of VEXAS syndrome.

In depth transcriptomic profiling defines a landscape of dysfunctional immune responses in patients with VEXAS syndrome.

Bilateral Alternate Orbital and Ocular Manifestations in a VEXAS Syndrome Patient.

Efficacy and safety of azacitidine for VEXAS syndrome: a large-scale retrospective study from FRENVEX.

The pathogenesis, clinical presentations and treatment of monogenic systemic vasculitis.

Real-world outcomes of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome at a tertiary referral centre.

Erythroid-stimulating agents in VEXAS syndrome: A retrospective study from an Italian multicentre cohort.

Bilateral Intraretinal Hemorrhages in VEXAS Syndrome.

VEXAS Syndrome: An Overlooked Threat.

Neutrophilic Urticarial Dermatosis: A Window into Systemic Inflammation and Autoimmune Disorders.

An Atypical Presentation of VEXAS Syndrome: A Diagnostic Conundrum.

VEXAS syndrome: A newly identified X-Linked hematoinflammatory disorder - A comprehensive overview of its genetic, molecular, inflammatory, and clinical landscape.

VEXAS Syndrome: Phenotype Alteration in the Long-Term Disease Duration.

Emerging treatment approaches for VEXAS syndrome: a systematic review and meta-analysis.

Case Report: Disseminated Nontuberculous Mycobacterial Infection in a VEXAS Syndrome Patient-Possible Association With Variant Allele Frequency.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome.

Clinical features and treatments of VEXAS syndrome in critical care: a scoping review.

Rapid Screening and Monitoring of UBA1 Mutations in VEXAS Syndrome.

New X for VEXAS: haploidentical allogeneic haematopoietic cell transplantation in VEXAS syndrome.

Inflammation and disrupted hematopoiesis drive clonal dominance in a mouse model of VEXAS syndrome.

Myeloid neoplasm inspired intensive therapy in VEXAS syndrome: A single-centre experience.

Mechanisms of hematopoietic clonal dominance in VEXAS syndrome.

A Case of VEXAS Syndrome Presenting with Unexplained Headache.

Therapeutic Challenges in the Management of VEXAS Syndrome: A Case Report.

The Challenging and Unique Diagnosis of VEXAS Syndrome: A Case Report.

Fatigue and chronic anaemia: Could it be VEXAS syndrome?

Histiocytoid Sweet Syndrome as a Manifestation of Myelodysplasia Cutis/Cutaneous VEXAS Syndrome.

[A case report of VEXAS syndrome].

Ocular Features of VEXAS Syndrome: A Systematic Review and Meta-analysis.

Is it VEXAS or is it vasculitis?

Systemic vasculitis: one year in review 2025.

[VEXAS- NEWLY REPORTED SYNDROME WITH OCULAR MANIFESTATIONS: CASE REPORT].

VEXAS syndrome: a Swiss national retrospective cohort study.

Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, and Somatic (VEXAS) Syndrome: A Diagnostic and Therapeutic Conundrum.

Efficacy and safety of conventional disease-modifying antirheumatic drugs in VEXAS syndrome: real-world data from the international AIDA network.

Kaposi sarcoma posthematopoietic stem cell transplant for vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome.

Altered IgG4 serum levels in VEXAS syndrome - a retrospective monocentric cohort study.

VEXAS syndrome with p.Met41Leu UBA1 gene mutation misdiagnosed as tumid lupus: A series of 3 cases.

Critical cardiovascular complications during non-immunosuppressive therapy for VEXAS syndrome.

VEXAS associated acute disseminated Encephalo-Myelitis (ADEM)-like syndrome: A case report and review of the literature.

[VEXAS syndrome - a chameleon of inflammatory syndromes].

Inflammatory Signatures in VEXAS Syndrome, Myelodysplasia Cutis, and Sweet Syndrome.

VEXAS Syndrome: A Perspective Focus on Genetics and Hematological Manifestations.

Aortitis in VEXAS Syndrome.

Efficacy and safety profile of biotechnological agents and Janus kinase inhibitors in VEXAS syndrome: data from the international AIDA Network VEXAS registry.

Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, and Somatic (VEXAS) Syndrome: A Case Report.

Decoding Cytoplasmic Vacuolization: Unraveling the Diagnostic Challenges of VEXAS Syndrome.

VEXAS syndrome through a rheumatologist's lens: insights from a Spanish national cohort.

This month in JAAD Case Reports: April 2025.

Neurological manifestations in patients with VEXAS syndrome.

Very long-term remission with azacitidine in VEXAS syndrome.

VEXAS, Chediak-Higashi syndrome and Danon disease: myeloid cell endo-lysosomal pathway dysfunction as a common denominator?

Improving outcomes in VEXAS syndrome: the need for prospective data.

VEXAS syndrome: is it more a matter of inflammation or hematopoietic clonality? A case series approach to diagnosis, therapeutic strategies and transplant management.

JAK2 is a critical therapeutic target in VEXAS syndrome treated with ruxolitinib.

A Clinicopathological Description of Kidney Features in VEXAS Syndrome.

A case of VEXAS syndrome with therapy refractive macular involvement.

Hypomethylating agents for patients with VEXAS without myelodysplastic syndrome: Clinical outcome and longitudinal follow-up of vacuolization and UBA1 clonal dynamics.