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. 2024 Jun;13(6):1479-1498.
doi: 10.1007/s40123-024-00916-z. Epub 2024 Mar 30.

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

Carla Gaggiano  1 , Abdurrahman Tufan  2 , Silvana Guerriero  3 , Gaafar Ragab  4   5 , Jurgen Sota  1 , Stefano Gentileschi  1 , Stefania Costi  6 , Ibrahim A Almaghlouth  7   8 , Andrea Hinojosa-Azaola  9 , Samar Tharwat  10 , Petros P Sfikakis  11   12 , Giuseppe Lopalco  13 , Matteo Piga  14 , Giovanni Conti  15 , George Fragoulis  11   12 , Angela Mauro  16   17 , Ezgi D Batu  18 , Seza Ozen  18 , Maria Tarsia  19 , Francesco La Torre  20 , Perla A Kawakami-Campos  21 , Antonio Vitale  1 , Valeria Caggiano  1 , Riza C Kardaş  22 , Gian Marco Tosi  23 , Bruno Frediani  1 , Tadej Avčin  24 , José Hernández-Rodríguez  25 , Luca Cantarini  26 , Claudia Fabiani  23 ; AIDA Network
Affiliations

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

Carla Gaggiano et al. Ophthalmol Ther. 2024 Jun.

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.

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Conflict of interest statement

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, Angela Mauro, Francesco La Torre, Ezgi Deniz Batu, Seza Ozen, Riza Can Kardaş, Maria Tarsia, Perla Ayumi Kawakami-Campos, George Fragoulis, Antonio Vitale, Valeria Caggiano, Gian Marco Tosi, Bruno Frediani, Tadej Avčin, José Hernández-Rodríguez, and Luca Cantarini declare that they have no competing interests. Claudia Fabiani is an Editorial Board member of Ophthalmology and Therapy and was not involved in the selection of peer reviewers for the manuscript nor any of the subsequent editorial decisions.

Figures

Fig. 1
Fig. 1
A diagram illustrating the patient selection process for the study as of the data lock on September 29, 2023. AIDA autoinflammatory diseases alliance
Fig. 2
Fig. 2
a Visualization of the distribution of age at Behçet’s disease (BD) onset and age at ocular involvement according to the anatomical classification of uveitis. b Anatomical classification of uveitis was different in male and female patients, with a distinct prevalence of posterior uveitis in male patients
Fig. 3
Fig. 3
Frequency of detection of pathological findings at the fundus fluorescein angiography. Percentages are calculated on the total number of procedures performed (19 procedures in 7 eyes)
Fig. 4
Fig. 4
a Visualization of the inverse correlation between age at the onset of Behçet’s disease (BD)-associated ocular manifestations and total Behçet’s disease Overall Damage Index (BODI) score at the end of the follow-up (Spearman ρ = –0.57; p < 0.01); b age at onset of BD-associated ocular inflammation was lower in the group of children who developed permanent changes in the anterior segment during the disease course
See this image and copyright information in PMC

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