Recurrent fever and association with severe organ involvement, mortality and treatment outcomes in VEXAS syndrome: data from the AIDA Network.

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Title: Recurrent fever and association with severe organ involvement, mortality and treatment outcomes in VEXAS syndrome: data from the AIDA Network.
Authors: Caggiano V; Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.; Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy., Sbalchiero J; Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.; Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy., Frassi M; Rheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, University of Brescia, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Brescia, Italy., Crisafulli F; Rheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, University of Brescia, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Brescia, Italy., Cavazzana I; Rheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, University of Brescia, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Brescia, Italy., Hinojosa-Azaola A; Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Martín-Nares E; Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Guaracha-Basañez GA; Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Torres-Ruiz J; Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Wiesik-Szewczyk E; Department of Internal Medicine, Pneumonology, Allergology, Clinical Immunology and Rare Diseases, Military Institute of Medicine, National Research Institute, Warsaw, Poland., Nowakowska-Plaza A; Department of Internal Medicine, Pneumonology, Allergology, Clinical Immunology and Rare Diseases, Military Institute of Medicine, National Research Institute, Warsaw, Poland., Sfriso P; Rheumatology Unit, Department of Medicine, University of Padua, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Padua, Italy., Bindoli S; Rheumatology Unit, Department of Medicine, University of Padua, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Padua, Italy., Rizzo S; Rheumatology Unit, Department of Medicine, University of Padua, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Padua, Italy., Hernández-Rodríguez J; Clinical Unit of Autoinflammatory Diseases, Department of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Hospital Clínic of Barcelona [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], University of Barcelona, Barcelona, Spain., Gómez-Caverzaschi V; Clinical Unit of Autoinflammatory Diseases, Department of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Hospital Clínic of Barcelona [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], University of Barcelona, Barcelona, Spain., Araújo O; Clinical Unit of Autoinflammatory Diseases, Department of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Hospital Clínic of Barcelona [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], University of Barcelona, Barcelona, Spain., Mayrink Giardini HA; Rheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de São Paulo, São Paulo, Brazil., González-García A; Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitario Ramón y Cajal, IRYCIS, Madrid, Spain., Lopalco G; Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy., Viapiana O; Rheumatology Unit, Department of Medicine, University and Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy., Bixio R; Rheumatology Unit, IRCCS Sacro Cuore Don Calabria Hospital, Negrar di Valpolicella, Verona, Italy., Tufan A; Department of Internal Medicine, Division of Rheumatology, Gazi University Hospital, Ankara, Türkiye., Hissaria P; Department of Clinical Immunology and Allergy, Royal Adelaide Hospital, Adelaide, SA, Australia.; Department of Immunopathology, SA Pathology, Adelaide, SA, Australia., Beecher M; Department of Clinical Immunology and Allergy, Royal Adelaide Hospital, Adelaide, SA, Australia.; Department of Immunopathology, SA Pathology, Adelaide, SA, Australia., Callisto A; Department of Clinical Immunology and Allergy, Royal Adelaide Hospital, Adelaide, SA, Australia.; Department of Immunopathology, SA Pathology, Adelaide, SA, Australia., De Paulis A; Department of Translational Medical Sciences, Section of Clinical Immunology, University of Naples Federico II, Naples, Italy.; Center for Basic and Clinical Immunology Research (CISI), World Allergy Organization (WAO) Center of Excellence, University of Naples Federico II, Naples, Italy., Dagna L; Division of Immunology, Transplants and Infectious Diseases, Università Vita-Salute San Raffaele, Milan, Italy.; Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS Ospedale San Raffaele, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Milan, Italy., Campochiaro C; Division of Immunology, Transplants and Infectious Diseases, Università Vita-Salute San Raffaele, Milan, Italy.; Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS Ospedale San Raffaele, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Milan, Italy., Tomelleri A; Division of Immunology, Transplants and Infectious Diseases, Università Vita-Salute San Raffaele, Milan, Italy.; Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS Ospedale San Raffaele, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Milan, Italy., Bugatti S; Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy.; Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Pavia, Italy., Milanesi A; Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy.; Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Pavia, Italy., Ruiz-Irastorza G; Faculty of Medicine and Nursery, University of the Basque Country, Euskal Herriko Unibertsitatea (UPV/EHU), Leioa, Biscay, Spain.; Autoimmune Diseases Unit, Biocruces Bizkaia Health Research Institute, Barakaldo, Biscay, Spain., Soto-Peleteiro A; Faculty of Medicine and Nursery, University of the Basque Country, Euskal Herriko Unibertsitatea (UPV/EHU), Leioa, Biscay, Spain.; Autoimmune Diseases Unit, Biocruces Bizkaia Health Research Institute, Barakaldo, Biscay, Spain., Piga M; Rheumatology Unit, Department of Medical Sciences and Public Health, University and Azienda Ospedaliero Universitaria (AOU) of Cagliari, Cagliari, Italy., Cauli A; Rheumatology Unit, Department of Medical Sciences and Public Health, University and Azienda Ospedaliero Universitaria (AOU) of Cagliari, Cagliari, Italy., Conti F; Department of Internal Medicine and Medical Specialties, Rheumatology Unit, Azienda Ospedaliero Universitaria (AOU) Policlinico Umberto I, Sapienza University of Rome, Rome, Italy., Cardamone C; Unità Operativa Complessa (UOC) of Internal Medicine - Rheumatology Outpatients Unit, Azienda Ospedaliero-Universitaria San Giovanni di Dio e Ruggi D'Aragona, Via San Leonardo 1, Salerno, Italy., Triggianese P; Unità Operativa Complessa (UOC) Medicina Interna - UOSD Geriatria, Università di Roma Tor Vergata, Rome, Italy.; Department of Biomedicine and Prevention, University of Rome Tor Vergata, Rome, Italy., Gurnari C; Department of Biomedicine and Prevention, University of Rome Tor Vergata, Rome, Italy.; Department of Translational Hematology and Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, United States., Vitetta R; Unit of Rheumatology, Azienda Sanitaria Locale di Vercelli (ASL VC) Sant' Andrea Hospital, Vercelli, Italy., Prete M; Rheumatic and Systemic Autoimmune Diseases Unit, Department of Interdisciplinary Medicine (DIM), University of Bari Medical School, Bari, Italy., Racanelli V; Centre for Medical Sciences, University of Trento and Internal Medicine Division, Santa Chiara Hospital, Provincial Health Care Agency (APSS), Trento, Italy., Talarico R; Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy., Ferro F; Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy., di Cianni F; Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy., Cuccaro MC; Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.; Hematology, Azienda Ospedaliera Universitaria Senese, University of Siena, Siena, Italy., Mayo-Juanatey A; Department of Rheumatology, Doctor Peset University Hospital, Valencia, Spain., Renieri A; Medical Genetics, Department of Medical Biotechnologies, University of Siena, Siena, Italy.; Department of Medical Biotechnologies, Med Biotech Hub and Competence Center, University of Siena, Siena, Italy.; Genetica Medica, Azienda Ospedaliero-Universitaria Senese, Siena, Italy., Sorrentino V; Molecular Medicine Section, Department of Molecular and Developmental Medicine, University of Siena, Siena, Italy., Ergun MA; Department of Medical Genetics, Faculty of Medicine, Gazi University, Ankara, Türkiye., Balistreri A; Bioengineering and Biomedical Data Science Lab, Department of Medical Biotechnologies, University of Siena, Siena, Italy., Frediani B; Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.; Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy., Bocchia M; Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.; Hematology, Azienda Ospedaliera Universitaria Senese, University of Siena, Siena, Italy., Fabiani C; Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.; Ophthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy., Cantarini L; Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.; Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy., Vitale A; Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.; Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.
Source: Frontiers in immunology [Front Immunol] 2026 Mar 13; Vol. 17, pp. 1753412. Date of Electronic Publication: 2026 Mar 13 (Print Publication: 2026).
Publication Type: Journal Article
Journal Info: Publisher: Frontiers Research Foundation] Country of Publication: Switzerland NLM ID: 101560960 Publication Model: eCollection Cited Medium: Internet ISSN: 1664-3224 (Electronic) Linking ISSN: 16643224 NLM ISO Abbreviation: Front Immunol Subsets: MEDLINE
Database: MEDLINE Ultimate
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