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

Antonio Vitale¹, Valeria Caggiano¹, Jessica Sbalchiero¹, Giuseppe Lopalco², Gaafar Ragab^{3

4}, Silvana Guerriero⁵, Ibrahim AlMaglouth⁶, Abdurrahman Tufan⁷, Roberto Giacomelli⁸, Haner Direskeneli⁹, Piero Ruscitti¹⁰, Gülen Hatemi¹¹, Francesco Carubbi^{12

13}, Ezgi Deniz Batu¹³, Seza Ozen¹⁴, Jurgen Sota¹, Henrique Ayres Mayrink Giardini¹⁵, Micol Frassi¹⁶, Petros P Sfikakis¹⁷, Federica Gatti², Claudia Ammoscato², Amina Maher^{3

4}, Ayman Abdel-Monem Ahmed Mahmoud^{3

4}, Rosanna Dammacco⁵, Hamit Kucuk⁷, Riza Can Kardas⁷, Ibrahim Yahya Cakir⁷, Fatma Alibaz Öner⁹, Gizem Sevik⁹, Martina Gentile¹⁰, Alican Karakoc¹¹, Alessia Alunno^{12

13}, Hulya Ercan Emreol¹⁴, Francesca Crisafulli¹⁶, Katerina Laskari¹⁷, Francesco Ciccia¹⁸, Maissa Thabet¹⁹, Naceur Feriel¹⁹, Serena Bugatti²⁰, Alessandra Milanesi²⁰, Maria Sole Chimenti²¹, Benedetta Monosi²¹, Matteo Piga²², Alberto Floris²³, Francesco Gavioli¹, Cecilia Beatrice Chighizola^{23

24}, José Hernández-Rodríguez²⁵, Marco Cattalini²⁶, Marcello Govoni²⁷, Ombretta Viapiana²⁸, Adele Civino²⁹, Daniela Opris-Belinski³⁰, Carla Gaggiano¹, Rosaria Talarico³¹, Annachiara Alemanno¹, Annarita Giardina³², Giacomo Emmi^{33

34

35}, Piercarlo Sarzi Puttini³⁶, Maria Cristina Maggio³⁷, Paola Parronchi³⁸, Piero Portincasa³⁹, Alejandra de-la-Torre⁴⁰, Laura Daniela Rodríguez-Camelo⁴⁰, Stefano Gentileschi¹, Angela Mauro^{41

42}, Gian Domenico Sebastiani⁴³, Alma Nunzia Olivieri⁴⁴, Ali Şahin⁴⁵, Donato Rigante^{46

47}, Emre Bilgin⁴⁸, Emanuela Del Giudice⁴⁹, Luciana Breda⁵⁰, Amato De Paulis^{51

52}, Alberto Lo Gullo⁵³, Şükran Erten⁵⁴, Samar Tharwat^{55

56}, Lampros Fotis⁵⁷, Armin Maier⁵⁸, Antonella Insalaco⁵⁹, Anastasios Karamanakos⁶⁰, Alessandro Conforti⁶¹, Özgül Soysal Gündüz⁶², Abdelhfeez Moshrif⁶³, Francesca Li Gobbi⁶⁴, Stefania Costi²⁴, Elena Bartoloni⁶⁵, Patrizia Barone⁶⁶, Serena Guiducci⁶⁷, Andrés Gonzáles-García⁶⁸, Inés Hernanz Rodriguez⁶⁹, Giovanni Conti⁷⁰, Annamaria Iagnocco⁷¹, Fatos Önen⁷², Sulaiman M Al-Mayouf⁷³, Didar Uçar⁷⁴, Alberto Balistreri⁷⁵, Bruno Frediani¹, Luca Cantarini⁷⁶, Claudia Fabiani⁷⁷

Affiliations

¹ Rheumatology 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.
² Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy.
³ Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt.
⁴ Faculty of Medicine, Newgiza University, 6th of October City, Giza, Egypt.
⁵ Department of Translational Biomedicine and Neuroscience (DiBraiN), University of Bari, Bari, Italy.
⁶ Rheumatology Unit, Department of Medicine, King Saud University, Riyadh, Saudi Arabia.
⁷ Division of Rheumatology, Department of Internal Medicine, Gazi University Hospital, Ankara, Turkey.
⁸ Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, Università Campus Bio-Medico Di Roma, Rome, Italy.
⁹ Department of Internal Medicine, Division of Rheumatology, School of Medicine, Marmara University, Istanbul, Turkey.
¹⁰ Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
¹¹ Department of Internal Medicine, Division of Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
¹² Behçet's Disease Research Center, Istanbul University-Cerrahpasa, Istanbul, Turkey.
¹³ Department 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.
¹⁴ Pediatric Rheumatology Unit, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey.
¹⁵ Rheumatology Division, Faculdade de Medicina, Hospital das Clinicas (HCFMUSP), Universidade de Sao Paulo, Sao Paulo, Brazil.
¹⁶ 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.
¹⁷ Joint 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.
¹⁸ Department of Precision Medicine, Università Degli Studi Della Campania Luigi Vanvitelli, Naples, Italy.
¹⁹ Internal Medicine Department, Farhat Hached University Hospital, Faculty of Medicine of Sousse, University of Sousse, Sousse, Tunisia.
²⁰ Department of Internal Medicine and Therapeutics, Università Di Pavia and Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
²¹ Rheumatology, Allergology and Clinical Immunology, Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.
²² Rheumatology Unit, Department of Medical Sciences, University and AOU of Cagliari, Cagliari, Italy.
²³ Pediatric Rheumatology Unit, Azienda Socio-Sanitaria Territoriale (ASST) Gaetano Pini Centro Specialistico Ortopedico Traumatologico (CTO), Milan, Italy.
²⁴ Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, University of Milan, Milan, Italy.
²⁵ Autoinflammatory Diseases Clinical Unit, Department of Autoimmune Diseases, Hospital Clinic of Barcelona, August Pi I Sunyer Biomedical Research Institute (IDIBAPS), University of Barcelona, Barcelona, Spain.
²⁶ Pediatric Clinic, University of Brescia and Spedali Civili Di Brescia [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], Brescia, Italy.
²⁷ Rheumatology Unit, Department of Medical Sciences, Azienda Ospedaliero-Universitaria S. Anna - Ferrara, University of Ferrara, Ferrara, Italy.
²⁸ Rheumatology Unit, Department of Medicine, University and Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy.
²⁹ Pediatric Rheumatology and Immunology Unit, Vito Fazzi Hospital, 73100, Lecce, Italy.
³⁰ Rheumatology and Internal Medicine Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
³¹ Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
³² UOC Medicina Interna, Ambulatorio Di Reumatologia, ARNAS Civico Di Cristina Benfratelli, Palermo, Italy.
³³ Department of Medical, Surgical and Health Sciences, University of Trieste, Trieste, Italy.
³⁴ Clinical Medicine and Rheumatology Unit, Cattinara University Hospital, Trieste, Italy.
³⁵ Centre for Inflammatory Diseases, Department of Medicine, Monash Medical Centre, Monash University, Clayton, VIC, Australia.
³⁶ Rheumatology Unit, Ospedale Sacco, Milan, Italy.
³⁷ University Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE) ``G. D'Alessandro'', University of Palermo, Palermo, Italy.
³⁸ Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
³⁹ Clinica 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.
⁴⁰ Neuroscience Research Group (NEUROS), Neurovitae Center for Neuroscience, Institute of Translational Medicine (IMT), School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia.
⁴¹ Department of Biomedical and Clinical Sciences, Fatebenefratelli Hospital, Università Di Milano, Milan, Italy.
⁴² Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, Milan, Italy.
⁴³ U.O.C. Reumatologia, Ospedale San Camillo-Forlanini, Rome, Italy.
⁴⁴ Department of Woman, Child and of General and Specialized Surgery, University of Campania ``Luigi Vanvitelli'', Naples, Italy.
⁴⁵ Division of Rheumatology, Department of Internal Medicine, Sivas Cumhuriyet University Medical Faculty, Sivas, Turkey.
⁴⁶ Department of Life Sciences and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
⁴⁷ Rare Diseases and Periodic Fevers Research Centre, Università Cattolica Sacro Cuore, Rome, Italy.
⁴⁸ Faculty of Medicine, Division of Rheumatology, Sakarya University, Sakarya, Turkey.
⁴⁹ Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Polo Pontino, Rome, Italy.
⁵⁰ Department of Paediatrics, University of Chieti-Pescara, Chieti, Italy.
⁵¹ Department of Translational Medical Sciences, Section of Clinical Immunology, University of Naples Federico II, Naples, Italy.
⁵² Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Naples, Italy.
⁵³ Unit of Rheumatology, Department of Medicine, ARNAS Garibaldi Hospital, Catania, Italy.
⁵⁴ Department of Rheumatology, Faculty of Medicine Ankara City Hospital, Ankara Yildirim Beyazit Universitesi, Ankara, Turkey.
⁵⁵ Rheumatology and Immunology Unit, Internal Medicine Department, Mansoura University, Mansoura, Egypt.
⁵⁶ Department of Internal Medicine, Faculty of Medicine, Horus University, New Damietta, Egypt.
⁵⁷ Department of Pediatrics, Attikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece.
⁵⁸ Rheumatology Unit, Department of Medicine, Central Hospital of Bolzano, Bolzano, Italy.
⁵⁹ Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Rome, Italy.
⁶⁰ Department of Rheumatology, "Evangelismos" General Hospital, Athens, Greece.
⁶¹ Ospedale San Paolo Di Civitavecchia, U.O. Medicina Generale, ASL Roma 4, Civitavecchia, Rome, Italy.
⁶² Division of Rheumatology, Department of Internal Medicine, School of Medicine, Manisa Celal Bayar University, Manisa, Turkey.
⁶³ Rheumatology Department, Faculty of Medicine, Al-Azhar University, Assiut, Egypt.
⁶⁴ Rheumatology Unit, Hospital S. Giovanni Di Dio, Azienda USL-Toscana Centro, Florence, Italy.
⁶⁵ Rheumatology Unit, Department of Medicine and Surgery, University of Perugia, Perugia, Italy.
⁶⁶ Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.
⁶⁷ Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
⁶⁸ Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitario Ramón y Cajal, IRYCIS, Madrid, Spain.
⁶⁹ Department of Ophthalmology, Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain.
⁷⁰ Pediatric Nephrology and Rheumatology Unit, Azienda Ospedaliero Universitaria (AOU) G Martino, Messina, Italy.
⁷¹ Academic Rheumatology Center, Dipartimento Scienze Cliniche e Biologiche, Università Degli Studi Di Torino, Turin, Italy.
⁷² School of Medicine, Department of Internal Medicine, Division of Rheumatology, Dokuz Eylül University, Izmir, Turkey.
⁷³ King Faisal Specialist Hospital and Research Center, College of Medicine, Department of Pediatrics, Alfaisal University, Riyadh, Saudi Arabia.
⁷⁴ Department of Ophthalmology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
⁷⁵ Bioengineering 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.
⁷⁶ Rheumatology 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.
⁷⁷ Ophthalmology 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.

PMID: 41205142
PMCID: PMC12882929
DOI: 10.1007/s40123-025-01258-0

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

Antonio Vitale et al. Ophthalmol Ther. 2026 Jan.

. 2026 Jan;15(1):223-237.

doi: 10.1007/s40123-025-01258-0. Epub 2025 Nov 8.

Authors

Affiliations

¹ Rheumatology 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.
² Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy.
³ Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt.
⁴ Faculty of Medicine, Newgiza University, 6th of October City, Giza, Egypt.
⁵ Department of Translational Biomedicine and Neuroscience (DiBraiN), University of Bari, Bari, Italy.
⁶ Rheumatology Unit, Department of Medicine, King Saud University, Riyadh, Saudi Arabia.
⁷ Division of Rheumatology, Department of Internal Medicine, Gazi University Hospital, Ankara, Turkey.
⁸ Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, Università Campus Bio-Medico Di Roma, Rome, Italy.
⁹ Department of Internal Medicine, Division of Rheumatology, School of Medicine, Marmara University, Istanbul, Turkey.
¹⁰ Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
¹¹ Department of Internal Medicine, Division of Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
¹² Behçet's Disease Research Center, Istanbul University-Cerrahpasa, Istanbul, Turkey.
¹³ Department 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.
¹⁴ Pediatric Rheumatology Unit, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey.
¹⁵ Rheumatology Division, Faculdade de Medicina, Hospital das Clinicas (HCFMUSP), Universidade de Sao Paulo, Sao Paulo, Brazil.
¹⁶ 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.
¹⁷ Joint 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.
¹⁸ Department of Precision Medicine, Università Degli Studi Della Campania Luigi Vanvitelli, Naples, Italy.
¹⁹ Internal Medicine Department, Farhat Hached University Hospital, Faculty of Medicine of Sousse, University of Sousse, Sousse, Tunisia.
²⁰ Department of Internal Medicine and Therapeutics, Università Di Pavia and Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
²¹ Rheumatology, Allergology and Clinical Immunology, Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.
²² Rheumatology Unit, Department of Medical Sciences, University and AOU of Cagliari, Cagliari, Italy.
²³ Pediatric Rheumatology Unit, Azienda Socio-Sanitaria Territoriale (ASST) Gaetano Pini Centro Specialistico Ortopedico Traumatologico (CTO), Milan, Italy.
²⁴ Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, University of Milan, Milan, Italy.
²⁵ Autoinflammatory Diseases Clinical Unit, Department of Autoimmune Diseases, Hospital Clinic of Barcelona, August Pi I Sunyer Biomedical Research Institute (IDIBAPS), University of Barcelona, Barcelona, Spain.
²⁶ Pediatric Clinic, University of Brescia and Spedali Civili Di Brescia [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], Brescia, Italy.
²⁷ Rheumatology Unit, Department of Medical Sciences, Azienda Ospedaliero-Universitaria S. Anna - Ferrara, University of Ferrara, Ferrara, Italy.
²⁸ Rheumatology Unit, Department of Medicine, University and Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy.
²⁹ Pediatric Rheumatology and Immunology Unit, Vito Fazzi Hospital, 73100, Lecce, Italy.
³⁰ Rheumatology and Internal Medicine Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
³¹ Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
³² UOC Medicina Interna, Ambulatorio Di Reumatologia, ARNAS Civico Di Cristina Benfratelli, Palermo, Italy.
³³ Department of Medical, Surgical and Health Sciences, University of Trieste, Trieste, Italy.
³⁴ Clinical Medicine and Rheumatology Unit, Cattinara University Hospital, Trieste, Italy.
³⁵ Centre for Inflammatory Diseases, Department of Medicine, Monash Medical Centre, Monash University, Clayton, VIC, Australia.
³⁶ Rheumatology Unit, Ospedale Sacco, Milan, Italy.
³⁷ University Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE) ``G. D'Alessandro'', University of Palermo, Palermo, Italy.
³⁸ Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
³⁹ Clinica 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.
⁴⁰ Neuroscience Research Group (NEUROS), Neurovitae Center for Neuroscience, Institute of Translational Medicine (IMT), School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia.
⁴¹ Department of Biomedical and Clinical Sciences, Fatebenefratelli Hospital, Università Di Milano, Milan, Italy.
⁴² Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, Milan, Italy.
⁴³ U.O.C. Reumatologia, Ospedale San Camillo-Forlanini, Rome, Italy.
⁴⁴ Department of Woman, Child and of General and Specialized Surgery, University of Campania ``Luigi Vanvitelli'', Naples, Italy.
⁴⁵ Division of Rheumatology, Department of Internal Medicine, Sivas Cumhuriyet University Medical Faculty, Sivas, Turkey.
⁴⁶ Department of Life Sciences and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
⁴⁷ Rare Diseases and Periodic Fevers Research Centre, Università Cattolica Sacro Cuore, Rome, Italy.
⁴⁸ Faculty of Medicine, Division of Rheumatology, Sakarya University, Sakarya, Turkey.
⁴⁹ Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Polo Pontino, Rome, Italy.
⁵⁰ Department of Paediatrics, University of Chieti-Pescara, Chieti, Italy.
⁵¹ Department of Translational Medical Sciences, Section of Clinical Immunology, University of Naples Federico II, Naples, Italy.
⁵² Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Naples, Italy.
⁵³ Unit of Rheumatology, Department of Medicine, ARNAS Garibaldi Hospital, Catania, Italy.
⁵⁴ Department of Rheumatology, Faculty of Medicine Ankara City Hospital, Ankara Yildirim Beyazit Universitesi, Ankara, Turkey.
⁵⁵ Rheumatology and Immunology Unit, Internal Medicine Department, Mansoura University, Mansoura, Egypt.
⁵⁶ Department of Internal Medicine, Faculty of Medicine, Horus University, New Damietta, Egypt.
⁵⁷ Department of Pediatrics, Attikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece.
⁵⁸ Rheumatology Unit, Department of Medicine, Central Hospital of Bolzano, Bolzano, Italy.
⁵⁹ Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Rome, Italy.
⁶⁰ Department of Rheumatology, "Evangelismos" General Hospital, Athens, Greece.
⁶¹ Ospedale San Paolo Di Civitavecchia, U.O. Medicina Generale, ASL Roma 4, Civitavecchia, Rome, Italy.
⁶² Division of Rheumatology, Department of Internal Medicine, School of Medicine, Manisa Celal Bayar University, Manisa, Turkey.
⁶³ Rheumatology Department, Faculty of Medicine, Al-Azhar University, Assiut, Egypt.
⁶⁴ Rheumatology Unit, Hospital S. Giovanni Di Dio, Azienda USL-Toscana Centro, Florence, Italy.
⁶⁵ Rheumatology Unit, Department of Medicine and Surgery, University of Perugia, Perugia, Italy.
⁶⁶ Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.
⁶⁷ Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
⁶⁸ Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitario Ramón y Cajal, IRYCIS, Madrid, Spain.
⁶⁹ Department of Ophthalmology, Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain.
⁷⁰ Pediatric Nephrology and Rheumatology Unit, Azienda Ospedaliero Universitaria (AOU) G Martino, Messina, Italy.
⁷¹ Academic Rheumatology Center, Dipartimento Scienze Cliniche e Biologiche, Università Degli Studi Di Torino, Turin, Italy.
⁷² School of Medicine, Department of Internal Medicine, Division of Rheumatology, Dokuz Eylül University, Izmir, Turkey.
⁷³ King Faisal Specialist Hospital and Research Center, College of Medicine, Department of Pediatrics, Alfaisal University, Riyadh, Saudi Arabia.
⁷⁴ Department of Ophthalmology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
⁷⁵ Bioengineering 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.
⁷⁶ Rheumatology 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.
⁷⁷ Ophthalmology 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.

PMID: 41205142
PMCID: PMC12882929
DOI: 10.1007/s40123-025-01258-0

Abstract

Introduction: Behçet'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.

Methods: Patients' data were collected in the International AutoInflammatory Disease Alliance (AIDA) Network registry dedicated to BD.

Results: A 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).

Conclusions: Major 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.

Keywords: AIDA Network; International registry; Rare diseases; Retinal vasculitis; Uveitis.

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Conflict of interest statement

Declarations. Conflict of Interest: The authors, therefore Antonio Vitale, Valeria Caggiano, Jessica Sbalchiero, Giuseppe Lopalco, Gaafar Ragab, Silvana Guerriero, Ibrahim AlMaglouth, Abdurraham Tufan, Roberto Giacomelli, Haner Direskeneli, Piero Ruscitti, Gülen Hatemi, Francesco Carubbi, Ezgi Deniz Batu, Seza Ozen, Jurgen Sota, Henrique Ayres Mayrink Giardini, Micol Frassi, Petros P Sfikakis, Federica Gatti, Claudia Ammoscato, Amina Maher, Ayman Abdel-Monem Ahmed Mahmoud, Rosanna Dammaco, Hamit Kucuk, Riza Can Kardas, Ibrahim Yahya Cakir, Fatma Alibaz Öner, Gizem Sevik, Martina Gentile, Alican Karakoc, Alessia Alunno, Hulya Ercan Emreol, Francesca Crisafulli, Katerina Laskari, Francesco Ciccia, Maissa Thabet, Naceur Feriel, Serena Bugatti, Alessandra Milanesi, Maria Sole Chimenti, Bendetta Monosi, Matteo Piga, Alberto Floris, Francesco Gavioli, Cecilia Beatrice Chighizola, José Hernández-Rodríguez, Marco Cattalini, Marcello Govoni, Ombretta Viapiana, Adele Civino, Daniela Opris-Belinski, Carla Gaggiano, Rosaria Talarico, Annachiara Alemanno, Annarita Giardina, Giacomo Emmi, Piercarlo Sarzi Puttini, Maria Cristina Maggio, Paola Parronchi, Piero Portincasa, Alejandra De-la-Torre, Laura Daniela Rodríguez-Camelo, Stefano Gentileschi, Angela Mauro, Gian Domenico Sebastiani, Alma Nunzia Olivieri, Ali Şahin, Donato Rigante, Emre Bilgin, Emanuela Del Giudice, Luciana Breda, Amato De Paulis, Alberto Lo Gullo, Şükran Erten, Samar Tharwat, Lampros Fotis, Armin Maier, Antonella Insalaco, Anastasios Karamanakos, Alessandro Conforti, Özgül Soysal Gündüz, Abdelhfeez Moshrif, Francesca Li Gobbi, Stefania Costi, Elena Bartoloni, Patrizia Barone, Serena Guiducci, Andrés Gonzáles-Garcia, Inés Hernanz Rodríguez, Giovanni Conti, Annamaria Iagnocco, Fatos Önen, Sulaiman M. Al-Mayouf, Didar Uçar, Alberto Balistreri, Bruno Frediani, Luca Cantarini, declare that the research was conducted without any commercial or financial relationships that could potentially create conflict of interest. Claudia Fabiani is an Editorial Board member of Ophthalmology and Therapy. She was not involved in the selection of peer reviewers for the manuscript nor any editorial decisions. Ethical Approval: The Ethics Committee of Azienda Ospedaliero-Universitaria Senese, Siena, Italy (Ref. N. 14,951; NCT05200715) approved the study, which was performed according to the Good Clinical Practice guidelines and the latest Declaration of Helsinki. Written informed consents for involved patients were collected at the time of patient enrollment in the AIDA registry and included permission to use data for research purposes. Clinical data are kept in accordance with the EU General Data Protection Regulations (GDPR), or other counterparts, on the processing of personal data and the protection of privacy (2016/679/EU).

References

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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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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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Conflict of interest statement

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