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; the AIDA Network

doi:10.1007/s40123-024-00916-z

Ophthalmology and Therapy (Mar 2024)

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,
the AIDA Network

Affiliations

Carla Gaggiano: 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”
Abdurrahman Tufan: Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Gazi University
Silvana Guerriero: Department of Ophthalmology and Otolaryngology, University of Bari
Gaafar Ragab: Rheumatology and Clinical Immunology Unit, Department of Internal Medicine, Faculty of Medicine, Cairo University
Jurgen Sota: 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”
Stefano Gentileschi: 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”
Stefania Costi: Unit of Pediatric Rheumatology, Azienda Socio-Sanitaria Territoriale (ASST) Gaetano Pini-Centro Specialistico Ortopedico Traumatologico (CTO)
Ibrahim A. Almaghlouth: Rheumatology Unit, Department of Medicine, College of Medicine, King Saud University
Andrea Hinojosa-Azaola: Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán
Samar Tharwat: Rheumatology and Immunology Unit, Internal Medicine Department, Mansoura University
Petros P. Sfikakis: 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
Giuseppe Lopalco: Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari
Matteo Piga: Rheumatology Unit, Department of Medical Sciences, University and AOU of Cagliari
Giovanni Conti: Pediatric Nephrology and Rheumatology Unit, Azienda Ospedaliero Universitaria (AOU) G Martino
George Fragoulis: 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
Angela Mauro: Department of Biomedical and Clinical Sciences, Fatebenefratelli Hospital, Università di Milano
Ezgi D. Batu: Department of Pediatric Rheumatology, Faculty of Medicine, Hacettepe University
Seza Ozen: Department of Pediatric Rheumatology, Faculty of Medicine, Hacettepe University
Maria Tarsia: 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]
Francesco La Torre: Department of Pediatrics, Giovanni XXIII Pediatric Hospital, University of Bari
Perla A. Kawakami-Campos: Department of Ophthalmology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán
Antonio Vitale: 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”
Valeria Caggiano: 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”
Riza C. Kardaş: Division of Rheumatology, Department of Internal Medicine, Gazi University Hospital
Gian Marco Tosi: 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]
Bruno Frediani: 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”
Tadej Avčin: 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]
José Hernández-Rodríguez: 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
Luca Cantarini: 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”
Claudia Fabiani: 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]
the AIDA Network

DOI: https://doi.org/10.1007/s40123-024-00916-z
Journal volume & issue: Vol. 13, no. 6
pp. 1479 – 1498

Abstract

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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.

Published in Ophthalmology and Therapy

ISSN: 2193-8245 (Print); 2193-6528 (Online)
Publisher: Adis, Springer Healthcare
Country of publisher: United Kingdom
LCC subjects: Medicine: Ophthalmology
Website: https://www.springer.com/journal/40123

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Abstract

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