Purpose To describe the disease characteristics and visual outcome of pediatric uveitis. Design Retrospective longitudinal observation. Participants 527 pediatric uveitis patients from the National Eye Institute, University of Illinois, Chicago and Oregon Health Sciences University. Methods Retrospective chart review. Main outcome measures Demographics, uveitis disease characteristics, complications, treatments, and visual outcomes and were determined at baseline and at 1, 3, 5 and 10 year time points. Results The patient population was 54 % female; 62.4% White, 12.5% Black, 2.7% Asian, 2.1 % multiracial and 14.61% Hispanic. Median age at diagnosis was 9.4 years. The leading diagnoses were idiopathic uveitis (28.8%), juvenile idiopathic arthritis-associated uveitis (20.9%), and pars planitis (17.1%). Insidious onset (58%) and persistent duration (75.3%) were most common. Anterior uveitis was predominant (44.6%). Complications were frequent, and cystoid macular edema (Odds Ratio: OR 2.94, p=0.006) and hypotony (OR 4.54, p=0.026) had the most significant visual impact. Ocular surgery was performed in 18.9% of patients. The prevalence of legal blindness was 9.23% at baseline, 6.52% at 1 year, 3.17% at 3 years, 15.15% at 5 years and 7.69% at 10 years. Posterior uveitis and panuveitis had more severe vision loss. Hispanic ethnicity was associated with a higher prevalence of infectious uveitis and vision loss at baseline. Conclusions The rate and spectrum of vision threatening complications of pediatric uveitis are significant. Prospective studies using standard outcome measures and including diverse populations are needed to identify children most at risk.

BS is associated with severe ocular and articular morbidity. Visceral involvement is common and may be life-threatening. Bone dysplastic changes may show diagnostic value and suggest a previously unknown role of NOD2 in bone morphogenesis. BS is resistant to current drugs, suggesting the need for novel targeted therapies.