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

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Gaggiano C., TUFAN A., Guerriero S., Ragab G., Sota J., Gentileschi S., ...More

Ophthalmology and Therapy, vol.13, no.6, pp.1479-1498, 2024 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Article
  • Volume: 13 Issue: 6
  • Publication Date: 2024
  • Doi Number: 10.1007/s40123-024-00916-z
  • Journal Name: Ophthalmology and Therapy
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, Directory of Open Access Journals
  • Page Numbers: pp.1479-1498
  • Keywords: Autoinflammatory diseases, Behçet’s disease, Paediatric ophthalmology, Rare disease registries, Retinal vasculitis, Uveitis
  • Gazi University Affiliated: Yes


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.