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

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.

• Authors: 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
• Journal: Ophthalmology and therapy (Ophthalmol Ther) (Mar 30 2024) ISSN: 2193-8245 [Print] England
• PMID: 38563868 (Publication Type: Journal Article)
• Copyright: © 2024. The Author(s).