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Purpose: To identify risk factors for a severe uveitis course among children with noninfectious uveitis., Design: Retrospective cohort study., Method: This was a retrospective analysis of a prospectively collected database. Records of 94 children with uveitis were reviewed at enrollment and every 3-6 months (2011-2015). Severe uveitis was defined as a history of ocular complications or a visual acuity (VA) of ≤20/200. Children were compared by disease, VA, complications, and race. Regression models were used to model risk factors for severe disease. When examining race, we focused on non-Hispanic African-American and non-Hispanic white children only., Results: Of 85 children with uveitis and complete ocular examinations, 27 (32%) had a history of a VA of ≤20/200. A subanalysis of non-Hispanic African-American and white children showed an increased prevalence of VA ≤20/200 in non-Hispanic African-Americans (18/25; 72% vs 4/43; 9%). Non-Hispanic African-Americans were more likely to be diagnosed at an older age (P = .030) and to have intermediate uveitis (P = .026), bilateral disease (P = .032), a history of VA ≤20/50 (P = .002), VA ≤20/200 (P < .001), and a higher rate of complications (P < .001). On multivariable analysis, non-Hispanic African-American race was a significant predictor of blindness (OR = 31.6, 95% CI 5.9-168.5, P < .001), after controlling for uveitis duration. Non-Hispanic African-Americans also developed 2.2 times more unique complications per year of disease than non-Hispanic whites when controlling for uveitis type and duration., Conclusions: There appear to be racial differences in the outcomes of children with uveitis. Non-Hispanic African-American children with non-juvenile idiopathic arthritis-associated uveitis may have worse visual outcomes with increased vision loss and ocular complications. These findings highlight the need for future studies in minority populations., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objective: Associations between shared epitope (SE)-encoding HLA-DRB1 alleles and rheumatoid arthritis (RA) are well established. However, only a limited number of studies have investigated these alleles in patients with childhood-onset RA, which is defined as rheumatoid factor- and/or anti-citrullinated protein antibody-positive juvenile idiopathic arthritis. The aims of this study were to investigate the largest cohort of patients with childhood-onset RA for association with SE alleles and to determine whether there is a hierarchy of risk based on the amino acid sequence of the SE., Methods: High-resolution HLA-DRB1 genotypes were obtained for 204 patients with childhood-onset RA and 373 healthy control subjects. Odds ratios (ORs) and 95% confidence intervals (95% CIs) were calculated for different SE-encoding HLA-DRB1 alleles. In addition, genotype ORs were calculated for combinations of SE alleles classified into S(2) , S(3P) , or L alleles, based on amino acid sequences in position 70-74 of the DRβ1 chain, as proposed by Tezenas du Montcel et al., Results: We confirmed associations between HLA-DRB1 SE alleles and childhood-onset RA (76% of patients carried 1 or 2 SE alleles compared with 46% of control subjects; OR 3.81, 95% CI 2.4-6.0, P < 1 × 10(-7) ). We also observed associations between individual SE alleles (HLA-DRB1*0101, *0401, *0404, *0405, *0408, and *1001) and childhood-onset RA. Genotype-specific risk estimates suggested a hierarchy of risk, with the highest risk among individuals heterozygous for S(2) /S(3P) (OR 22.3, 95% CI 9.9-50.5, P < 0.0001)., Conclusion: We confirm the association between SE-encoding HLA-DRB1 alleles and susceptibility to childhood-onset RA. The excess risk conferred by carriage of the combination of S(2) and S(3P) risk alleles suggests that children with DRβ1 chains containing the KRAA and QRRAA or RRRAA sequences are especially susceptible to RA., (Copyright © 2012 by the American College of Rheumatology.)

Objective: To determine the validity and reliability of a novel questionnaire to measure vision-related quality of life (VRQOL) in children ages 8-18 years for use in juvenile idiopathic arthritis (JIA)-associated uveitis: the Effects of Youngsters' Eyesight on Quality of Life (EYE-Q)., Methods: Several steps validated the EYE-Q. We interviewed experts and children on how vision affects a child's activities. We developed new items and selected relevant items from existing instruments. We administered initial versions of the EYE-Q to normal-sighted children and those with JIA-associated uveitis. For this study, children with various (or no) ocular conditions were recruited from a clinical population. Visual acuity and contrast sensitivity were performed, and the EYE-Q and Pediatric Quality of Life Inventory (PedsQL) were administered. The EYE-Q was repeated 10 days later. Patients, parents, and physicians rated vision severity., Results: Of 120 patients, 48% were female, 46.7% had no visual impairment, and 53.3% had bilateral eye involvement. The mean age was 11.3 years. There were significant differences in the measures based on visual acuity (P < 0.001). Children with more severe visual acuity and bilateral eye involvement had worse EYE-Q scores (P < 0.001). There were significant associations between the EYE-Q and PedsQL (r = 0.375), repeat EYE-Q (r = 0.864), and clinical measures of ocular disease (r = -0.620)., Conclusions: Our study provides evidence of the validity and reliability of the EYE-Q in the measurement of VRQOL. The EYE-Q may complement clinical measures of visual impairment and overall QOL and become an important tool in the assessment of QOL in JIA-associated uveitis., (Copyright © 2011 by the American College of Rheumatology.)