GuidelineArthritis & rheumatology (Hoboken, N.J.)2026
American College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel.
Abstract readPractice Guideline
In one paragraph
Guideline in Arthritis & rheumatology (Hoboken, N.J.), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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24citing papers in PubMed
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Who cites it
24 citing papers in PubMed.
- Myelodysplasia in VEXAS syndrome.Blood advances · 2026Review
- VEXAS Syndrome: Clues to Avoid Vexation.Journal of cutaneous pathology · 2026Review
- Diagnostic and Therapeutic Challenge: VEXAS Syndrome with Multisystem Inflammation and Myelodysplasia: A Case Report.Mediterranean journal of rheumatology · 2026Article
- An Italian cartography of VEXAS-related thrombosis.HemaSphere · 2026Article
- SomaticBlood neoplasia · 2026Article
- Review
- Clinical characteristics and a screening tool for VEXAS syndrome: a case-control study from China.Orphanet journal of rare diseases · 2026Article
- Monogenic autoimmune and autoinflammatory disorders in adulthood: recent discoveries and implications for rheumatology practice.Rheumatology (Oxford, England) · 2026Review
- 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: Clinical Features, Hematologic Involvement, and Clinical Outcomes of Current and Emerging Therapies.Hematology reports · 2026Review
- VEXAS syndrome.Nature reviews. Disease primers · 2026Review
- Navigating through uncertainty-Experience from the UK national VEXAS MDT.British journal of haematology · 2026Article
- 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
- Establishing a consensus definition of VEXAS flare for clinical research.Rheumatology (Oxford, England) · 2026Article
- Rapid cutaneous remission response to tofacitinib and systemic corticosteroids in VEXAS syndrome with a novel UBA1 mutation.JAAD case reports · 2026Article
- A rapid, accessible real-time PCR approach to identify UBA1 somatic mutations in VEXAS syndrome.Frontiers in medicine · 2026Article
- The First Reported Case of VEXAS Syndrome in Lebanon: Efficacy of Azacitidine as a Therapeutic Option-Case Report.Case reports in hematology · 2026Article
- The profile of clinical and laboratory features of Chinese VEXAS syndrome patients with hematological abnormalities: a single-center case series.Frontiers in immunology · 2026Article
- VEXAS Syndrome for the laboratory physician: a case report.Frontiers in immunology · 2026Article
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Authors and funding
59 authors.
Arsene MekinianService de médecine interne, Hôpital Saint Antoine, Assistance Publique Hopitaux de Paris, Sorbonne Université, Imagine Institute, CEREMAIAA reference center, Paris, France.ORCID 0000-0003-2849-3049
Sophie Georgin-LavialleInternal Medicine Department, Hopital Tenon, Assistance Publique Hopitaux de Paris, Sorbonne University, CEREMAIAA reference center, Paris, France.
Marcela A FerradaDivision of Rheumatology and Immunology, Department of Medicine, Baltimore, University of Maryland, USA.
Sinisa SavicLeeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom.
Matthew J KosterDivision of Rheumatology, Mayo Clinic, Rochester, MN, USA.ORCID 0000-0002-2895-6755
Olivier KosmiderUniversité de Paris Cité, Institut Cochin, CNRS UMR8104, INSERM U1016, Paris, France.
Thibault ComontService de Médecine interne, Centre Hospitalier Universitaire de Toulouse, Université Toulouse III-Paul Sabatier Faculté de santé, Pole IUC de Toulouse Oncopole CHU, Toulouse, France.
Mael HeibligService d'Hématologie Clinique, Hospices Civils de Lyon, Hôpital Lyon Sud, Pierre Bénite, France.
Juan I ArosteguiDepartment of Immunology, Hospital Clínic, Barcelona, Spain.
Annmarie BoscoHematology Department, Prince of Wales Hospital, Sydney, New South Wales, Australia.
Rim BourguibaDepartment of Internal Medicine, Hôpital des forces de sécurité de l'intérieur, Tunis, Tunisia.ORCID 0000-0002-7352-9074
Katherine R CalvoDepartment of Laboratory Medicine, National Institutes of Health, Bethesda, MD, USA.
Catherine CargoHematological Malignancy Diagnostic Service, Leeds Cancer Centre, St James's University Hospital, Leeds, United Kingdom.
Chiara CattaneoASST Spedali Civili of Brescia, University of Brescia, Brescia, Italy.
François ChassetDermatology and Allergology Department, Assistance Publique Hopitaux de Paris, Sorbonne University, Paris, France.
Henrique CoelhoDepartment of Hematology, Centro Hospitalar Vila Nova de Gaia, Porto, Portugal.
Corrado CampochiaroUnit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS Ospedale San Raffaele & Vita-Salute San Raffaele University, Milan, Italy.ORCID 0000-0001-6806-3794
Francesca CrisafulliASST Spedali Civili of Brescia, University of Brescia, Brescia, Italy.ORCID 0000-0003-0319-1538
Stephanie Ducharme-BenardService de médecine interne, Hôpital du Sacré-Cœur de Montréal, Montréal, Québec, Canada.
Raquel FariaUnidade de Imunologia Clínica, Unidade Local de Saúde Santo António (ULSSA), Porto, Portugal.
Franco FranceschiniRheumatology and Clinical Immunology, ASST Spedali Civili of Brescia and Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy.
Micol FrassiRheumatology and Clinical Immunology, ASST Spedali Civili of Brescia and Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy.
Emma M GroarkeHematology Branch, National Heart, Lung, Blood Institute, National Institute of Health, Bethesda, MD, USA.
Carmelo GurnariDepartment of Translational Hematology and Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA.ORCID 0000-0001-6829-5544
Yervand HakobyanDepartment of Adult Hematology, Yeolyan Hematology Center, Yerevan, Armenia; Department of Hematology and Transfusion Medicine, National Institute of Health, Yerevan, Armenia.
Yvan JamillouxService de médecine interne, Hôpital de la Croix Rousse, Hospices Civils de Lyon, Lyon, France.ORCID 0000-0001-5249-3650
Ciprian JurcutDepartment of Internal Medicine, Dr. Carol Davila Central Military Emergency University Hospital, Bucharest, 010825, Romania.
Yohei KirinoDepartment of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, Yokohama, Japan.ORCID 0000-0002-9488-661X
Austin KulasekararajDepartment of Hematology, Kings College Hospital and Kings College London, London, UK.
Hiroyoshi KunimotoDepartment of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
Lauren M MadiganDepartment of Dermatology, University of Utah, Salt Lake City, Utah.
Heřman F MannInstitute of Rheumatology and Department of Rheumatology, First Faculty of Medicine, Charles University, Prague, Czech Republic.
Chiara MarvisiRheumatology Unit, Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Reggio Emilia, Italy.ORCID 0000-0001-7027-1410
Marcin MilchertDepartment of Internal Diseases Rheumatology Diabetology Geriatrics and Clinical Immunology with the Sub-Department of Gastroenterology, Pomeranian Medical University in Szczecin, Szczecin, Poland.D43:N68.ORCID 0000-0002-0943-8768
Sara MoraisCentro Hospitalar Universitário de Santo António, Porto, Portugal.
Katja SockelMedical Clinic and Policlinic I, University Hospital Dresden, TU Dresden, Dresden, Germany.
Francesco MuratoreUniversity of Modena and Reggio Emilia, Modena, Italy.
Hideaki NakajimaDepartment of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
Mrinal M PatnaikDivision of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA.
Luísa RegadasCentro Hospitalar e Universitário do Porto, Porto, Portugal.
Marie RobinService d'hématologie-greffe, Hôpital Saint-Louis, Assistance Publique Hopitaux de Paris, Université de Paris Cité, Paris, France.
Abraham RutgersDepartment of Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, 9713, GZ, Groningen, The Netherlands.ORCID 0000-0002-1641-6890
Carlo SalvaraniUniversity of Modena and Reggio Emilia, Modena, Italy.ORCID 0000-0003-3708-3148
Anthony M SammelPrince of Wales Clinical School, University of New South Wales, Sydney, New South Wales, Australia.
Joerg SeebachDivision of Clinical Immunology and Allergy, Department of Medical Specialties, University Hospital and Faculty of Medicine, Geneva, Switzerland.
Pierre SujobertLaboratory of Hematology, Hôpital Lyon Sud, Hospices Civils de Lyon, Pierre Bénite, Lyon, France.
Alessandro TomelleriUnit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS Ospedale San Raffaele & Vita-Salute San Raffaele University, Milan, Italy.ORCID 0000-0002-5440-2078
Geoffrey UrbanskiDepartment of Orofacial Sciences, School of Dentistry, University of California, San Francisco, CA, USA.ORCID 0000-0001-5719-8423
Frédéric VandergheynstDepartment of Internal Medicine, Hôpitaux Universitaires de Bruxelles, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium.
Romana VieiraRheumatology Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Vila Nova de Gaia, Portugal.
David S ViswanathaDivision of Hematopathology, Department of Pathology and Laboratory Medicine, Mayo Clinic, Rochester, MN, USA.
Ewa Więsik-SzewczykDepartment of Internal Medicine, Pneumonology, Allergology and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, National Health Institute, Warsaw, Poland.
Elisa DiralUnit of Hematology and Bone Marrow Transplant, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Benjamin TerrierService de Médecine interne, Hôpital Cochin, Assistance Publique Hopitaux de Paris, Université Paris Cité, Paris, France.ORCID 0000-0001-6612-7336
Bhavisha A PatelHematology Branch, National Heart, Lung, Blood Institute, National Institute of Health, Bethesda, MD, USA.
Pierre FenauxService de Hématologie, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France.
Peter C GraysonVasculitis Translational Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.ORCID 0000-0002-8269-9438
David B BeckCenter for Human Genetics and Genomics, New York University School of Medicine, New York, NY, USA.ORCID 0000-0002-5884-6231
International VEXAS working group, and with endorsement of EuroBloodNet, the European Reference Network in Rare Hematological Diseases
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveVacuoles E1 enzyme X-linked autoinflammatory somatic syndrome (VEXAS) is a recently identified rare genetic disorder associated with somatic mutations in the UBA1 gene. VEXAS presents with a combination of inflammatory and hematologic manifestations, leading to increased morbidity and mortality.
methodsGiven the variability in disease presentation and the limited number of studies to date, no clinical documents currently exist to provide guidance to health care providers about the management of VEXAS. To address this gap, we formed an international multidisciplinary panel of VEXAS experts.
resultsThrough formalized meetings and a voting process, the group developed consensus clinical guidance considerations for the management of VEXAS. These considerations offer practical advice on several key topics: (1) clinical features of VEXAS, (2) UBA1 screening methods, (3) the diagnosis of myelodysplastic syndromes (MDSs) in patients with VEXAS, and (4) prognosis and management. The aim is to provide expert guidance on which patients to test, how to test for VEXAS, how to approach MDS in the context of VEXAS, and considerations for management.
conclusionThis work marks the first formal international consensus guidance for VEXAS and is intended to be used as a resource for clinicians seeking to understand the disease and its management.
Indexed as
Hereditary Autoinflammatory DiseasesMyelodysplastic SyndromesUbiquitin-Activating EnzymesConsensusHumansMutationRheumatologySkin Diseases, GeneticUBA1 protein, humanUbiquitin-Activating Enzymes
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