Ocular Manifestations in Juvenile Behçet's Disease: A Registry-Based Analysis from the AIDA Network

Carla Gaggiano; Abdurrahman Tufan; Silvana Guerriero; Gaafar Ragab; Jurgen Sota; Stefano Gentileschi; Stefania Costi; Ibrahim A Almaghlouth; Andrea Hinojosa-Azaola; Samar Tharwat; Petros P Sfikakis; Giuseppe Lopalco; Matteo Piga; Giovanni Conti; George Fragoulis; Angela Mauro; Ezgi D Batu; Seza Ozen; Maria Tarsia; Francesco La Torre; Perla A Kawakami-Campos; Antonio Vitale; Valeria Caggiano; Riza C Kardaş; Gian Marco Tosi; Bruno Frediani; Tadej Avčin; José Hernández-Rodríguez; Luca Cantarini; Claudia Fabiani; AIDA Network

doi:10.1007/s40123-024-00916-z

Ocular Manifestations in Juvenile Behçet's Disease: A Registry-Based Analysis from the AIDA Network

Ophthalmol Ther. 2024 Mar 30. doi: 10.1007/s40123-024-00916-z. Online ahead of print.

Authors

Carla Gaggiano¹, Abdurrahman Tufan², Silvana Guerriero³, Gaafar Ragab^{4

5}, Jurgen Sota¹, Stefano Gentileschi¹, Stefania Costi⁶, Ibrahim A Almaghlouth^{7

8}, Andrea Hinojosa-Azaola⁹, Samar Tharwat¹⁰, Petros P Sfikakis^{11

12}, Giuseppe Lopalco¹³, Matteo Piga¹⁴, Giovanni Conti¹⁵, George Fragoulis^{11

12}, Angela Mauro^{16

17}, Ezgi D Batu¹⁸, Seza Ozen¹⁸, Maria Tarsia¹⁹, Francesco La Torre²⁰, Perla A Kawakami-Campos²¹, Antonio Vitale¹, Valeria Caggiano¹, Riza C Kardaş²², Gian Marco Tosi²³, Bruno Frediani¹, Tadej Avčin²⁴, José Hernández-Rodríguez²⁵, Luca Cantarini²⁶, Claudia Fabiani²³; AIDA Network

Affiliations

¹ Rheumatology 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], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
² Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Gazi University, Ankara, Turkey.
³ Department of Ophthalmology and Otolaryngology, University of Bari, Bari, Italy.
⁴ Rheumatology and Clinical Immunology Unit, Department of Internal Medicine, Faculty of Medicine, Cairo University, Giza, Egypt.
⁵ Faculty of Medicine, New Giza University, Giza, Egypt.
⁶ Unit of Pediatric Rheumatology, Azienda Socio-Sanitaria Territoriale (ASST) Gaetano Pini-Centro Specialistico Ortopedico Traumatologico (CTO), Milan, Italy.
⁷ Rheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
⁸ College of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
⁹ Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
¹⁰ Rheumatology and Immunology Unit, Internal Medicine Department, Mansoura University, Mansoura, Egypt.
¹¹ First Department of Propaedeutic Internal Medicine, Medical School, Laiko General Hospital [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], National and Kapodistrian University of Athens, Athens, Greece.
¹² Joint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
¹³ Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy.
¹⁴ Rheumatology Unit, Department of Medical Sciences, University and AOU of Cagliari, Cagliari, Italy.
¹⁵ Pediatric Nephrology and Rheumatology Unit, Azienda Ospedaliero Universitaria (AOU) G Martino, Messina, Italy.
¹⁶ Department of Biomedical and Clinical Sciences, Fatebenefratelli Hospital, Università di Milano, Milan, Italy.
¹⁷ Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, Milan, Italy.
¹⁸ Department of Pediatric Rheumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
¹⁹ Clinical Pediatrics, Department of Molecular Medicine and Development, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.
²⁰ Department of Pediatrics, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.
²¹ Department of Ophthalmology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
²² Division of Rheumatology, Department of Internal Medicine, Gazi University Hospital, Ankara, Turkey.
²³ 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.
²⁴ Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University of Ljubljana and University Medical Centre Ljubljana [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Ljubljana, Slovenia.
²⁵ Department of Autoimmune Diseases, Hospital Clínic of Barcelona [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
²⁶ Rheumatology 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], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy. cantariniluca@hotmail.com.

PMID: 38563868
DOI: 10.1007/s40123-024-00916-z

Abstract

Introduction: This study aims to characterize ocular manifestations of juvenile Behçet's disease (jBD).

Methods: This was a registry-based observational prospective study. All subjects with jBD from the Autoinflammatory Diseases Alliance (AIDA) Network BD Registry showing ocular manifestations before 18 years were enrolled.

Results: We included 27 of 1000 subjects enrolled in the registry (66.7% male patients, 45 affected eyes). The median (interquartile range [IQR]) age at ocular involvement was 14.2 (4.7) years. Uveitis affected 91.1% of eyes (anterior 11.1%, posterior 40.0%, panuveitis 40.0%), retinal vasculitis 37.8% and other manifestations 19.8%. Later onset (p = 0.01) and male predominance (p = 0.04) characterized posterior involvement. Ocular complications occurred in 51.1% of eyes. Patients with complications had earlier onset (p < 0.01), more relapses (p = 0.02) and more prolonged steroidal treatment (p = 0.02). The mean (standard deviation [SD]) central macular thickness (CMT) at the enrolment and last visit was 302.2 (58.4) and 293.3 (78.2) μm, respectively. Fluorescein angiography was pathological in 63.2% of procedures, with a mean (SD) Angiography Scoring for Uveitis Working Group (ASUWOG) of 17.9 (15.5). At the last visit, ocular damage according to the BD Overall Damage Index (BODI) was documented in 73.3% of eyes. The final mean (SD) best corrected visual acuity (BCVA) logMAR was 0.17 (0.47) and blindness (BCVA logMAR < 1.00 or central visual field ≤ 10°) occurred in 15.6% of eyes. At multivariate regression analysis, human leukocyte antigen (HLA)-B51 + independently predicted a + 0.35 change in the final BCVA logMAR (p = 0.01), while a higher BCVA logMAR at the first assessment (odds ratio [OR] 5.80; p = 0.02) independently predicted blindness.

Conclusions: The results of this study may be leveraged to guide clinical practice and future research on this rare sight-threatening condition.

Keywords: Autoinflammatory diseases; Behçet’s disease; Paediatric ophthalmology; Rare disease registries; Retinal vasculitis; Uveitis.