| Title: | Ocular manifestations in juvenile Behçet’s disease : a registry-based analysis from the AIDA network |
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| Authors: | ID Gaggiano, Carla (Author)
ID Tufan, Abdurrahman (Author)
ID Guerriero, Silvana (Author)
ID Ragab, Gaafar Mohamed (Author)
ID Sota, Jurgen (Author)
ID Gentileschi, Stefano (Author)
ID Costi, Stefania (Author)
ID Almaghlouth, Ibrahim A (Author)
ID Hinojosa-Azaola, Andrea (Author)
ID Tharwat, Samar (Author)
ID Avčin, Tadej (Author), et al. |
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| Files: |  PDF - Presentation file, download (1,00 MB)
MD5: 12EC993BA221D7F729E641171B970277
 URL - Source URL, visit https://link.springer.com/article/10.1007/s40123-024-00916-z
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| Language: | English |
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| Typology: | 1.01 - Original Scientific Article |
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| Organization: |  UKC LJ - Ljubljana University Medical Centre
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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. |
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| Keywords: | autoinflammatory diseases, Behçet’s disease, paediatric ophthalmology, rare disease registries, retinal vasculitis, uveitis |
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| Publication status: | Published |
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| Publication version: | Version of Record |
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| Year of publishing: | 2024 |
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| Number of pages: | str. 1479-1498 |
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| Numbering: | Vol 13 |
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| PID: | 20.500.12556/DiRROS-30114  |
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| UDC: | 617.7 |
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| ISSN on article: | 2193-6528 |
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| DOI: | 10.1007/s40123-024-00916-z |
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| COBISS.SI-ID: | 205656835 |
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| Note: | Nasl. z nasl. zaslona;
Opis vira z dne 28. 8. 2024;
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| Publication date in DiRROS: | 15.06.2026 |
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| Views: | 34 |
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| Downloads: | 21 |
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