20.500.12556/DiRROS-30114 Ocular manifestations in juvenile Behçet’s disease a registry-based analysis from the AIDA network 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. autoinflammatory diseases Behçet’s disease paediatric ophthalmology rare disease registries retinal vasculitis uveitis true false true Angleški jezik Ni določen Neznano 2026-06-15 14:34:39 2026-06-15 14:34:39 2026-06-16 03:54:51 0000-00-00 00:00:00 2024 0 0 Nasl. z nasl. zaslona; Opis vira z dne 28. 8. 2024; str. 1479-1498 Vol 13 2024 0000-00-00 Zaloznikova Objavljeno NiDoloceno 0000-00-00 0000-00-00 0000-00-00 617.7 2193-6528 10.1007/s40123-024-00916-z 205656835 RAZ_Gaggiano_Carla_2024.pdf RAZ_Gaggiano_Carla_2024.pdf 1 12EC993BA221D7F729E641171B970277 3471b758e946a6fd5aecd04b215a83f3db209ba10bfea6f42c8107f2ad27b85b c61e8d7e-68b6-11f1-aebd-001a4af901a5 https://dirros.openscience.si/Dokument.php?lang=slv&id=45477 https://link.springer.com/article/10.1007/s40123-024-00916-z 0 d9256447-68b6-11f1-aebd-001a4af901a5 https://dirros.openscience.si/Dokument.php?lang=slv&id=45478 Univerzitetni klinični center Ljubljana 0 0 0