ArticleOphthalmology and therapy2026
The Risk of Developing Ocular Involvement Among Behçet's Disease Patients Presenting with Mucocutaneous Involvement at Disease Onset: Data from the International AIDA Network Behçet's Disease Registry.
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- Timing matters: diagnostic delay and major organ involvement in Behçet's syndrome.Rheumatology (Oxford, England) · 2026Article
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99 authors.
Antonio VitaleRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences 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], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Valeria CaggianoRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences 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], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Jessica SbalchieroRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences 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], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Giuseppe LopalcoDepartment of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy.
Gaafar RagabInternal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt.
Silvana GuerrieroDepartment of Translational Biomedicine and Neuroscience (DiBraiN), University of Bari, Bari, Italy.
Ibrahim AlMaglouthRheumatology Unit, Department of Medicine, King Saud University, Riyadh, Saudi Arabia.
Abdurrahman TufanDivision of Rheumatology, Department of Internal Medicine, Gazi University Hospital, Ankara, Turkey.
Roberto GiacomelliRheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, Università Campus Bio-Medico Di Roma, Rome, Italy.
Haner DireskeneliDepartment of Internal Medicine, Division of Rheumatology, School of Medicine, Marmara University, Istanbul, Turkey.
Piero RuscittiRheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
Gülen HatemiDepartment of Internal Medicine, Division of Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Francesco CarubbiBehçet's Disease Research Center, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Ezgi Deniz BatuDepartment of Life, Health & Environmental Sciences, Internal Medicine and Nephrology Division, ASL1 Avezzano-Sulmona-L'Aquila, San Salvatore Hospital, University of L'Aquila, L'Aquila, Italy.
Seza OzenPediatric Rheumatology Unit, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey.
Jurgen SotaRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences 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], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Henrique Ayres Mayrink GiardiniRheumatology Division, Faculdade de Medicina, Hospital das Clinicas (HCFMUSP), Universidade de Sao Paulo, Sao Paulo, Brazil.
Micol FrassiRheumatology 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.
Petros P SfikakisJoint Academic Rheumatology Program, National and Kapodistrian University of Athens Medical School, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Athens, Greece.
Federica GattiDepartment of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy.
Claudia AmmoscatoDepartment of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy.
Amina MaherInternal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt.
Ayman Abdel-Monem Ahmed MahmoudInternal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt.
Rosanna DammaccoDepartment of Translational Biomedicine and Neuroscience (DiBraiN), University of Bari, Bari, Italy.
Hamit KucukDivision of Rheumatology, Department of Internal Medicine, Gazi University Hospital, Ankara, Turkey.
Riza Can KardasDivision of Rheumatology, Department of Internal Medicine, Gazi University Hospital, Ankara, Turkey.
Ibrahim Yahya CakirDivision of Rheumatology, Department of Internal Medicine, Gazi University Hospital, Ankara, Turkey.
Fatma Alibaz ÖnerDepartment of Internal Medicine, Division of Rheumatology, School of Medicine, Marmara University, Istanbul, Turkey.
Gizem SevikDepartment of Internal Medicine, Division of Rheumatology, School of Medicine, Marmara University, Istanbul, Turkey.
Martina GentileRheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
Alican KarakocDepartment of Internal Medicine, Division of Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Alessia AlunnoBehçet's Disease Research Center, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Hulya Ercan EmreolPediatric Rheumatology Unit, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey.
Francesca CrisafulliRheumatology 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.
Katerina LaskariJoint Academic Rheumatology Program, National and Kapodistrian University of Athens Medical School, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Athens, Greece.
Francesco CicciaDepartment of Precision Medicine, Università Degli Studi Della Campania Luigi Vanvitelli, Naples, Italy.
Maissa ThabetInternal Medicine Department, Farhat Hached University Hospital, Faculty of Medicine of Sousse, University of Sousse, Sousse, Tunisia.
Naceur FerielInternal Medicine Department, Farhat Hached University Hospital, Faculty of Medicine of Sousse, University of Sousse, Sousse, Tunisia.
Serena BugattiDepartment of Internal Medicine and Therapeutics, Università Di Pavia and Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Alessandra MilanesiDepartment of Internal Medicine and Therapeutics, Università Di Pavia and Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Maria Sole ChimentiRheumatology, Allergology and Clinical Immunology, Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.
Benedetta MonosiRheumatology, Allergology and Clinical Immunology, Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.
Matteo PigaRheumatology Unit, Department of Medical Sciences, University and AOU of Cagliari, Cagliari, Italy.
Alberto FlorisPediatric Rheumatology Unit, Azienda Socio-Sanitaria Territoriale (ASST) Gaetano Pini Centro Specialistico Ortopedico Traumatologico (CTO), Milan, Italy.
Francesco GavioliRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences 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], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Cecilia Beatrice ChighizolaPediatric Rheumatology Unit, Azienda Socio-Sanitaria Territoriale (ASST) Gaetano Pini Centro Specialistico Ortopedico Traumatologico (CTO), Milan, Italy.
José Hernández-RodríguezAutoinflammatory Diseases Clinical Unit, Department of Autoimmune Diseases, Hospital Clinic of Barcelona, August Pi I Sunyer Biomedical Research Institute (IDIBAPS), University of Barcelona, Barcelona, Spain.
Marco CattaliniPediatric Clinic, University of Brescia and Spedali Civili Di Brescia [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], Brescia, Italy.
Marcello GovoniRheumatology Unit, Department of Medical Sciences, Azienda Ospedaliero-Universitaria S. Anna - Ferrara, University of Ferrara, Ferrara, Italy.
Ombretta ViapianaRheumatology Unit, Department of Medicine, University and Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy.
Adele CivinoPediatric Rheumatology and Immunology Unit, Vito Fazzi Hospital, 73100, Lecce, Italy.
Daniela Opris-BelinskiRheumatology and Internal Medicine Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
Carla GaggianoRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences 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], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Rosaria TalaricoRheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
Annachiara AlemannoRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences 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], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Annarita GiardinaUOC Medicina Interna, Ambulatorio Di Reumatologia, ARNAS Civico Di Cristina Benfratelli, Palermo, Italy.
Giacomo EmmiDepartment of Medical, Surgical and Health Sciences, University of Trieste, Trieste, Italy.
Piercarlo Sarzi PuttiniRheumatology Unit, Ospedale Sacco, Milan, Italy.
Maria Cristina MaggioUniversity Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE) ``G. D'Alessandro'', University of Palermo, Palermo, Italy.
Paola ParronchiDepartment of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
Piero PortincasaClinica Medica ``A. Murri'', Division of Internal Medicine, Department of Precision and Regenerative Medicine and Ionian Area (DiMePre-J), University of Bari Aldo Moro, Bari, Italy.
Alejandra de-la-TorreNeuroscience Research Group (NEUROS), Neurovitae Center for Neuroscience, Institute of Translational Medicine (IMT), School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia.
Laura Daniela Rodríguez-CameloNeuroscience Research Group (NEUROS), Neurovitae Center for Neuroscience, Institute of Translational Medicine (IMT), School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia.
Stefano GentileschiRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences 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], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Angela MauroDepartment of Biomedical and Clinical Sciences, Fatebenefratelli Hospital, Università Di Milano, Milan, Italy.
Gian Domenico SebastianiU.O.C. Reumatologia, Ospedale San Camillo-Forlanini, Rome, Italy.
Alma Nunzia OlivieriDepartment of Woman, Child and of General and Specialized Surgery, University of Campania ``Luigi Vanvitelli'', Naples, Italy.
Ali ŞahinDivision of Rheumatology, Department of Internal Medicine, Sivas Cumhuriyet University Medical Faculty, Sivas, Turkey.
Donato RiganteDepartment of Life Sciences and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
Emre BilginFaculty of Medicine, Division of Rheumatology, Sakarya University, Sakarya, Turkey.
Emanuela Del GiudiceDepartment of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Polo Pontino, Rome, Italy.
Luciana BredaDepartment of Paediatrics, University of Chieti-Pescara, Chieti, Italy.
Amato De PaulisDepartment of Translational Medical Sciences, Section of Clinical Immunology, University of Naples Federico II, Naples, Italy.
Alberto Lo GulloUnit of Rheumatology, Department of Medicine, ARNAS Garibaldi Hospital, Catania, Italy.
Şükran ErtenDepartment of Rheumatology, Faculty of Medicine Ankara City Hospital, Ankara Yildirim Beyazit Universitesi, Ankara, Turkey.
Samar TharwatRheumatology and Immunology Unit, Internal Medicine Department, Mansoura University, Mansoura, Egypt.
Lampros FotisDepartment of Pediatrics, Attikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece.
Armin MaierRheumatology Unit, Department of Medicine, Central Hospital of Bolzano, Bolzano, Italy.
Antonella InsalacoDivision of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Rome, Italy.
Anastasios KaramanakosDepartment of Rheumatology, "Evangelismos" General Hospital, Athens, Greece.
Alessandro ConfortiOspedale San Paolo Di Civitavecchia, U.O. Medicina Generale, ASL Roma 4, Civitavecchia, Rome, Italy.
Özgül Soysal GündüzDivision of Rheumatology, Department of Internal Medicine, School of Medicine, Manisa Celal Bayar University, Manisa, Turkey.
Abdelhfeez MoshrifRheumatology Department, Faculty of Medicine, Al-Azhar University, Assiut, Egypt.
Francesca Li GobbiRheumatology Unit, Hospital S. Giovanni Di Dio, Azienda USL-Toscana Centro, Florence, Italy.
Stefania CostiDepartment of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, University of Milan, Milan, Italy.
Elena BartoloniRheumatology Unit, Department of Medicine and Surgery, University of Perugia, Perugia, Italy.
Patrizia BaroneDepartment of Clinical and Experimental Medicine, University of Catania, Catania, Italy.
Serena GuiducciDivision of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
Andrés Gonzáles-GarcíaSystemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitario Ramón y Cajal, IRYCIS, Madrid, Spain.
Inés Hernanz RodriguezDepartment of Ophthalmology, Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain.
Giovanni ContiPediatric Nephrology and Rheumatology Unit, Azienda Ospedaliero Universitaria (AOU) G Martino, Messina, Italy.
Annamaria IagnoccoAcademic Rheumatology Center, Dipartimento Scienze Cliniche e Biologiche, Università Degli Studi Di Torino, Turin, Italy.
Fatos ÖnenSchool of Medicine, Department of Internal Medicine, Division of Rheumatology, Dokuz Eylül University, Izmir, Turkey.
Sulaiman M Al-MayoufKing Faisal Specialist Hospital and Research Center, College of Medicine, Department of Pediatrics, Alfaisal University, Riyadh, Saudi Arabia.
Didar UçarDepartment of Ophthalmology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Alberto BalistreriBioengineering and Biomedical Data Science Lab, Department of Medical Biotechnologies, University of Siena, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], Siena, Italy.
Bruno FredianiRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences 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], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Luca CantariniRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences 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], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy. cantariniluca@hotmail.com.ORCID http://orcid.org/0000-0002-7352-1275
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
introductionBehçet's disease (BD) frequently arises with exclusively mucocutaneous involvement, but some patients will develop major organ involvement, including ocular inflammation. This study aims to assess the patients' demographic and clinical characteristics that may be associated with the development of ocular involvement in patients with BD with exclusively mucocutaneous involvement in the early stages.
methodsPatients' data were collected in the International AutoInflammatory Disease Alliance (AIDA) Network registry dedicated to BD.
resultsA total of 328 patients with BD were enrolled, 36 (11%) of whom developed ocular involvement over time. The following variables were significantly associated with the development of ocular inflammation at binary logistic regression: positive family history for BD (OR 4.32, 95% CI 1.16-14.72, p = 0.02), Arab ethnicity (OR 3.6, 95% CI 1.3-9.9, p = 0.01), presence of major oral aphthous ulceration (OR 3.26, 95% CI 1.18-8.99, p = 0.02), pseudofolliculitis plus erythema nodosum (OR 4.58, 95% CI 1.33-15.7, p = 0.016); conversely, white patients/patients of European descent (OR 0.33, 95% CI 0.12-0.91, p = 0.03) and the presence of a low number (one to two) ulcers at genital aphthous flares (OR 0.002, 95% CI ~ 0-0.07, p = 0.0006) were protective against the occurrence of ocular inflammatory manifestations. Genital aphthosis with more than five concurrent ulcers was associated with eye involvement in Arab patients (OR 209.2, 95% CI 5.8-7497, p = 0.003).
conclusionsMajor oral aphthosis, genital aphthous attacks with more than five concurrent ulcers, erythema nodosum coexisting with pseudofolliculitis, a positive family history of BD, and Arab ethnicity are associated with a higher risk of ocular involvement when BD arises with the sole mucocutaneous involvement.
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AIDA NetworkInternational registryRare diseasesRetinal vasculitisUveitis
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