ArticleFrontiers in immunology2026
Ophthalmic involvement in VEXAS syndrome and its influence on mortality: insights from the international AIDA network registry.
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Article in Frontiers in immunology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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47 authors.
Jurgen SotaRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], Siena, Italy.
Andrea Hinojosa-AzaolaDepartment of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Eduardo Martín-NaresDepartment of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Paolo SfrisoRheumatology Unit, Department of Medicine, University of Padua, Padua, Italy.
Sara BindoliRheumatology Unit, Department of Medicine, University of Padua, Padua, Italy.
Pravin HissariaDepartment of Clinical Immunology and Allergy, Royal Adelaide Hospital, Adelaide, SA, Australia.
Mark BeecherDepartment of Clinical Immunology and Allergy, Royal Adelaide Hospital, Adelaide, SA, Australia.
José Hernández-RodríguezAutoinflammatory Diseases Clinical Unit, Department of Autoimmune Diseases, Hospital Clinic of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Center of the European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA), Barcelona, Spain.
Verónica Gómez-CaverzaschiAutoinflammatory Diseases Clinical Unit, Department of Autoimmune Diseases, Hospital Clinic of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Center of the European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA), Barcelona, Spain.
Micol FrassiRheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center, University of Brescia, Brescia, Italy.
Francesca CrisafulliRheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center, University of Brescia, Brescia, Italy.
Lorenzo DagnaDivision of Immunology, Transplants and Infectious Diseases, Università Vita-Salute San Raffaele, Milan, Italy.
Corrado CampochiaroUnit of Immunology, Rheumatology, Allergy and Rare Diseases, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Ospedale San Raffaele, European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center, Milan, Italy.
Serena BugattiRheumatology Department, Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. Matteo Fondazione, University of Pavia, Pavia, Italy.
Alessandra MilanesiRheumatology Department, Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. Matteo Fondazione, University of Pavia, Pavia, Italy.
Guillermo Ruiz-IrastorzaFaculty of Medicine and Nursery, University of the Basque Country, UPV/EHU, Leioa, Biscay, Spain.
Adriana Soto-PeleteiroFaculty of Medicine and Nursery, University of the Basque Country, UPV/EHU, Leioa, Biscay, Spain.
Matteo PigaRheumatology Unit, Department of Medical Sciences and Public Health, University and Azienda Opsedaliero-Universitaria (AOU) of Cagliari, Cagliari, Italy.
Ombretta ViapianaRheumatology Unit, Department of Medicine, University and Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy.
Abdurrahman TufanDivision of Rheumatology, Department of Internal Medicine, Gazi University Hospital, Ankara, Türkiye.
Ertugrul Cagri BolekDivision of Rheumatology, Department of Internal Medicine, Gazi University Hospital, Ankara, Türkiye.
Paola TriggianeseUnità Operativa Complessa (UOC) Medicina Interna - Unità Operativa Semplice Dipartimentale (UOSD) Geriatria, Università di Roma Tor Vergata, Rome, Italy.
Marcella PreteInternal Medicine Unit, Department of Interdisciplinary Medicine, University of Bari "Aldo Moro", Bari, Italy.
Jessica SblachieroRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], Siena, Italy.
Valeria CaggianoRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], Siena, Italy.
Antonio VitaleRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], Siena, Italy.
Henrique Ayres Mayrink GiardiniRheumatology Division, Faculdade de Medicina, Hospital das Clinicas (HCFMUSP), Universidade de Sao Paulo, Sao Paulo, Brazil.
Giuseppe LopalcoDepartment of Precision and Rigenerative Medicine and Ionian Area (DiMePRe-J), Polyclinic Hospital, University of Bari, Bari, Italy.
Fabrizio ContiRheumatology Unit, Department of Clinical Internal, Anesthesiologic and Cardiovascular Sciences, Sapienza University of Rome, Rome, Italy.
Paolo MoscatoDepartment of Medicine, University Hospital San Giovanni di Dio e Ruggi d'Aragona, Salerno, Italy.
Chiara CardamoneDepartment of Medicine, University Hospital San Giovanni di Dio e Ruggi d'Aragona, Salerno, Italy.
Ewa Wiesik-SzewczyDepartment of Internal Medicine, Pneumonology, Allergology and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, National Research Institute, Warsaw, Poland.
Andrés González-GarcíaSystemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitario Ramón y Cajal, IRYCIS, Madrid, Spain.
Amato De PaulisDepartment of Translational Medical Sciences, Section of Clinical Immunology, University of Naples Federico II, Naples, Italy.
Rosetta VitettaUnit of Rheumatology, Azienda Sanitaria Locale Vercelli (ASL VC) Sant' Andrea Hospital, Vercelli, Italy.
Perla Ayumi Kawakami-CamposDepartment of Ophthalmology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Alessandra BrancaleoniDepartment of Ophthalmology, Azienda Unità Sanitaria Locale (AUSL) Romagna - Ospedale di Rimini, Rimini, Italy.
Andrea MercantiDepartment of Ophthalmology, Azienda Unità Sanitaria Locale (AUSL) Romagna - Ospedale di Rimini, Rimini, Italy.
Alessandra RenieriMedical Genetics, Department of Medical Biotechnologies, University of Siena, Siena, Italy.
Monica BocchiaHematology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Siena, Italy.
Gaafar RagabRheumatology and Clinical Immunology Unit, Internal Medicine Department, Faculty of Medicine, Cairo University, Giza, Egypt.
Vishali GuptaAdvanced Eye Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Alejandra de-la-TorreNeuroscience Research Group (NEUROS), NeuroVitae Center, Escuela de Medicina y Ciencias de la Salud, Universidad del Rosario, Bogotá, Colombia.
Bruno FredianiRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], Siena, Italy.
Carmelo GurnariDepartment of Biomedicine and Prevention, University of Rome Tor Vergata, Rome, Italy.
Luca CantariniRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], Siena, Italy.
Claudia FabianiOphthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], Siena, Italy.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Orbital inflammation is the most common presentation of VEXAS, with any orbital structure potentially affected. Non-sight-threatening and uncomplicated anterior non-granulomatous uveitis and anterior diffuse scleritis follow in frequency, along with episcleritis. Ophthalmic involvement was significantly associated with relapsing polychondritis (
Indexed as
Polychondritis, RelapsingScleritisUveitis, AnteriorFemaleHumansMaleMyelodysplastic SyndromesPrognosisRegistriesSkin Diseases, GeneticSyndromeinflammatory orbitopathymortalityscleritisuveitisVEXAS syndrome
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