ArticleFrontiers in immunology2026
Risk of intestinal involvement in mucocutaneous-onset Behçet's disease: data from the AIDA network registry.
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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, 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, 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, Siena, 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, 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 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, Türkiye.
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, Türkiye.
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, Türkiye.
Francesco CarubbiUniversity of L'Aquila, Department of Life, Health & Environmental Sciences, Internal Medicine and Nephrology Division, ASL1 Avezzano-Sulmona-L'Aquila, San Salvatore Hospital, L'Aquila, Italy.
Ezgi Deniz BatuPediatric Rheumatology Unit, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Türkiye.
Seza OzenPediatric Rheumatology Unit, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Türkiye.
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, 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, Brescia, Italy.
Petros P SfikakisJoint Academic Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece.
Maria MorroneDepartment of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy.
Federica GattiDepartment 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 of Translational Biomedicine and Neuroscience (DiBraiN), University of Bari, Bari, Italy.
Hamit KucukDivision of Rheumatology, Department of Internal Medicine, Gazi University Hospital, Ankara, Türkiye.
Riza Can KardasDivision of Rheumatology, Department of Internal Medicine, Gazi University Hospital, Ankara, Türkiye.
Ibrahim Yahya CakirDivision of Rheumatology, Department of Internal Medicine, Gazi University Hospital, Ankara, Türkiye.
Letizia Pia di CorciaRheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, Università Campus Bio-Medico di Roma, Rome, Italy.
Fatma Alibaz ÖnerDepartment of Internal Medicine, Division of Rheumatology, School of Medicine, Marmara University, Istanbul, Türkiye.
Gizem SevikDepartment of Internal Medicine, Division of Rheumatology, School of Medicine, Marmara University, Istanbul, Türkiye.
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, Türkiye.
Alessia AlunnoUniversity of L'Aquila, Department of Life, Health & Environmental Sciences, Internal Medicine and Nephrology Division, ASL1 Avezzano-Sulmona-L'Aquila, San Salvatore Hospital, L'Aquila, Italy.
Hulya Ercan EmreolPediatric Rheumatology Unit, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Türkiye.
Francesca CrisafulliRheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy.
Maria TektonidouJoint Academic Rheumatology Program, National and Kapodistrian University of Athens Medical School, 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.
Serena BugattiDepartment of Internal Medicine and Therapeutics, Università di Pavia and Division of Rheumatology, Fondazione Istituto di ricovero ecura a carattere scientifico (IRCCS) Policlinico San Matteo, Pavia, Italy.
Alessandra MilanesiDepartment of Internal Medicine and Therapeutics, Università di Pavia and Division of Rheumatology, Fondazione Istituto di ricovero ecura a carattere scientifico (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 FlorisRheumatology Unit, Department of Medical Sciences, University and AOU of Cagliari, Cagliari, Italy.
Andrea Hinojosa-AzaolaDepartment of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas Y Nutrición Salvador Zubirán, Mexico City, Mexico.
Guillermo Arturo Guaracha-BasañezDepartment of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas Y Nutrición Salvador Zubirán, Mexico City, Mexico.
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, 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, 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, Siena, Italy.
Rosaria TalaricoRheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
Annarita GiardinaUOC Medicina Interna, Ambulatorio di Reumatologia, Azienda ospedaliera di rilievo nazionale e di alta specializzazione (ARNAS) Civico Di Cristina Benfratelli, Palermo, Italy.
Giacomo EmmiDepartment of Medical, Surgical and Health Sciences, and Clinical Medicine and Rheumatology Unit, Cattinara University Hospital, 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.
Juanita Cardona-LópezNeuroscience 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, Siena, Italy.
Angela MauroDepartment of Biomedical and Clinical Sciences, Fatebenefratelli Hospital, Università di Milano, Milan, Italy.
Gian Domenico SebastianiUnità operativa complessa (U.O.C.) Reumatologia, Ospedale San Camillo-Forlanini, Rome, Italy.
Maria Francesca GicchinoDepartment 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, Türkiye.
Donato RiganteDepartment of Life Sciences and Public Health, Fondazione Policlinico Universitario A. Gemelli Istituto di ricovero e cura a carattere scientifico (IRCCS), Rome, Italy.
Emre BilginDivision of Rheumatology, Faculty of Medicine, Sakarya University, Sakarya, Türkiye.
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, Azienda ospedaliera di rilievo nazionale e di alta specializzazione (ARNAS) Garibaldi Hospital, Catania, Italy.
Şükran ErtenDepartment of Rheumatology, Faculty of Medicine Ankara City Hospital, Ankara Yildirim Beyazit Universitesi, Ankara, Türkiye.
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.
Maier ArminRheumatology Unit, Department of Medicine, Central Hospital of Bolzano, Bolzano, Italy.
Antonella InsalacoDivision of Rheumatology, Ospedale Pediatrico Bambino Gesù, Istituto di ricovero ecura a carattere scientifico (IRCCS), Rome, Italy.
Anastasios KaramanakosDepartment of Rheumatology, "Evangelismos" General Hospital, Athens, Greece.
Alessandro ConfortiOspedale San Paolo di Civitavecchia, U.O. Medicina Generale, ASL Roma 4, Rome, Italy.
Özgül Soysal GündüzDivision of Rheumatology, Department of Internal Medicine, School of Medicine, Manisa Celal Bayar University, Manisa, Türkiye.
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 González-GarcíaSystemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitario Ramón y Cajal, Istituto Ramon y Cajal de Investigation Sanitaria (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 ÖnenDepartment of Internal Medicine, Division of Rheumatology, School of Medicine, Dokuz Eylül University, Izmir, Türkiye.
Sulaiman M Al-MayoufDepartment of Pediatrics, King Faisal Specialist Hospital and Research Center, College of Medicine, Alfaisal University, Riyadh, Saudi Arabia.
Ali İbrahim HatemiDepartment of Internal Medicine, Division of Gastroenterology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Instabul, Türkiye.
Alberto BalistreriBioengineering and Biomedical Data Science Lab, Department of Medical Biotechnologies, University of Siena, Siena, Italy.
Claudia FabianiOphthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese, 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, Siena, Italy.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Objective: Behçet's disease (BD) may initially manifest solely with mucocutaneous involvement. This study aimed to identify demographic and clinical factors associated with subsequent intestinal involvement in patients presenting exclusively with mucocutaneous manifestations during early disease stages. Methods: Data were obtained from the International AutoInflammatory Disease Alliance Network registry dedicated to BD; a Bayesian statistical approach was employed to address the limited sample size resulting from subgroup stratifications. Results: In total, 328 BD patients with exclusively mucocutaneous onset were enrolled; of these, 46 (14%) developed intestinal involvement over time. The risk of ocular involvement was higher among patients with intestinal manifestations (OR: 3.02, 95% CrI: 1.24-6.08; posterior probability: 99.3%). Minor aphthous ulcers without major aphthosis were protective towards intestinal involvement (OR: 0.47, 95% CrI: 0.22-0.98; posterior probability: 97.98%). Conversely, major aphthous ulcers increased the risk (OR: 3.25, 95% CrI: 1.50-7.02; posterior probability: 99.7%), along with the combination of oral major aphthosis with: i) genital aphthosis (OR = 2.77, 95%CrI: 1.14-6.58; posterior probability: 98.45%); ii) pseudofolliculitis (OR = 3.18, 95%CrI: 1.15-8.33; posterior probability: 98.72%); iii) genital aphthosis plus pseudofolliculitis (OR = 5.22, 95%CrI: 1.71-16.35; posterior probability of 99.77%). Pseudofolliculitis plus cutaneous manifestations other than erythema nodosum were protective against intestinal involvement (OR = 0.01, 95%CrI: 0.0-0.98; posterior probability: 97.55%). Conclusion: Major aphthosis was the strongest factor associated with intestinal involvement in BD patients initially presenting with mucocutaneous symptoms only. In such patients, intestinal involvement correlated with increased risk of ocular inflammation.
Indexed as
Behcet SyndromeIntestinal DiseasesAdultFemaleHumansMaleMiddle AgedRegistriesRisk FactorsStomatitis, AphthousYoung Adultgutinflammationprecision medicinepredictorsrisk factors
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