Ocular manifestations in juvenile Behçet’s disease: a registry-based analysis

Ocular inflammation represents one of the three cardinal manifestations of Behçet’s disease (BD) along with recurrent oral and genital ulceration. This study aims to characterize ocular manifestations of BD in the pediatric age through an in-depth analysis of data from a large international cohort, the AIDA Network Behçet’s disease registry. This is a registry-based observational prospective analysis. All subjects with juvenile (j)BD enrolled in the registry showing ocular inflammatory manifestations before the age of 18 years were included. The study cohort included 27 children (66.7% males). The mean (SD) age at onset was 11.9 (3.9) years. The median (IQR) age at the onset of ocular manifestations was 14.2 (4.7) years. Ocular manifestations were present at onset in 20 subjects (74.1%). There was an inverse correlation between age at BD onset and delay of ocular involvement (Pearson’s r= -0.43, p=0.02). The HLA-B51 haplotype was present in 14 subjects (51.9%). Ocular inflammation manifested bilaterally in 18 children (66.7%), affecting a total of 45 eyes. Uveitis was found in 39 eyes (86.7%), retinal vasculitis in 17 (37.8%), retinitis in 6 (13.3%), retrobulbar optic neuritis in 2 (4.4%), and papillitis in 1 (2.2%). Uveitis was classified anatomically as anterior in 11.1%, posterior in 40.0%, or panuveitis in 40.0% of eyes. Median age at ocular involvement was higher in children with posterior uveitis than in those with anterior uveitis or panuveitis (p=0.04; Dunn’s post-hoc: p=0.02 for panuveitis and p=0.09 for anterior uveitis). Posterior uveitis was observed in 53.3% of males and 18.2% of females (p=0.04), anterior uveitis showed a tendency towards female sex (27.3% versus 6.7%, p=0.07). Ocular complications occurred in 23 eyes (51.1%), the most frequent being cataract (28.9% of eyes), macular edema (20.0%) and posterior synechiae (15.6%). Patients with complications had lower age at ocular involvement (p<0.01), higher number of relapses (p=0.02) and more prolonged treatment with systemic corticosteroids (CS) (p=0.02). The presence of complications was associated with structural changes in the anterior or posterior segment (p<0.01). The mean (SD) central macular thickness measured by optical coherent tomography at the enrolment and at the last follow-up were 302.2 (58.4) and 293.3 (78.2) micron, respectively. Fundus fluorescein angiography identified pathological signs in 12/19 procedures (63.2%), with a mean (SD) ASUWOG score of 17.9 (15.5). Patients were treated with systemic CS in 20 out of 27 cases (74.1%), with a mean (SD) higher dosage of prednisone of 0.5 (0.3) mg/kg/day and a median (IQR) treatment duration of 17.0 (23.5) months. Azathioprine (44.4%), cyclosporin A (33.3%), methotrexate (22.2%), adalimumab (63.0%) and infliximab (25.9%) were the most common therapeutic choices. At the end of the follow-up, a median (IQR) BODI score of 1.5 (3.3) was calculated; it inversely correlated with the age at ocular involvement (Spearman’s r=-0.5681, p<0.01) and it was higher in children with bilateral ocular disease (p=0.02). In addition, signs of ocular damage were documented in 33 eyes (73.3%). As for the visual prognosis, a median (IQR) BCVA of 1.0 (0.5) was measured and blindness occurred in 7 eyes (15.6%) of 6 children. According to multivariate regression analysis, the presence of HLA-B51 may significantly predict a reduction of -0.3 of the BCVA at the last follow-up (p=0.01). Also, the BCVA at the initial evaluation (OR 0.59; p<0.01) and the presence of posterior synechiae (OR 1.62; p=0.01) were independent predictors of unilateral or bilateral blindness. This research offers an exhaustive overview of ocular manifestations related to BD in children, drawing from an international registry cohort. Considering the rarity of this condition and the specific focus of the study, this in-depth analysis contributes significant insights into current literature, which may be leveraged for potentially shaping clinical approaches and inspiring future research.