Outcomes of children with juvenile idiopathic arthritis-associated uveitis

项目摘要 幼年特发性关节炎相关性葡萄膜炎（JIA-U）是一种炎症性眼病， 患有JIA的儿童可导致白内障、青光眼、视力丧失和失明。现行筛查指南 旨在识别JIA中有眼疾风险的儿童。然而，儿科风湿病学家和 眼科医生对葡萄膜炎进行全面筛查的能力是有限的，因为指南 尚未更新以纳入所有幼年型关节炎亚型和其他已知的视力限制风险因素 JIA的并发症筛查JIA和其他相关的儿童自身免疫性疾病至关重要， 早期葡萄膜炎检测可以预防视觉并发症并影响治疗管理。同样地， 必要时早期积极治疗可避免严重的视力并发症。改善健康 结果和JIA-U儿童的生活质量，我们需要更好地了解葡萄膜炎的危险因素， 长期视力结果，以及视力和身体残疾对儿童生活的影响。早期和 通过鉴定葡萄膜炎的易感性和严重程度的危险因素， 结果测量的标准化，以及HLA危险等位基因的意义的阐明将提高 评价甲减的影响，了解疾病的病因、发病机制和转归。 同样，一个改进的风险分层方案可以减少不必要的眼科就诊， 确定儿童患严重眼病和并发症的风险更大。 本研究的目的是：1）确定JIA-U在临床特征和病程方面的差异， 欧洲和非洲血统的JIA儿童患者，以确定临床和遗传风险标志物， 葡萄膜炎，2）检查在一项为期4年的前瞻性研究中确定的风险因素， 以确定哪些因素与葡萄膜炎的最终发展相关，并提供数据 3）确定关节炎和葡萄膜炎对身体和视觉的影响 残疾、视力特异性QOL和使用主观和客观测量的总体QOL。使用 综合生物学、遗传学和心理社会学的方法，我们希望开发出更广泛适用的风险 基于JIA亚型的分层方案，并确定新的风险因素，以确定 最有可能患严重残疾和失明的儿童，以便制定干预措施， 他们的总体结果。 这位候选人是埃默里大学的儿科风湿病学家和儿科助理教授。她 已获得临床研究理学硕士学位。她致力于临床结果的职业生涯 儿童自身免疫性眼病的研究。她组建了一个出色的指导团队， 在儿科风湿病学、儿科眼科学、儿科流行病学、结果研究和 这与他们的目标有关。这一指导奖将帮助她成为一个成功的 独立临床科学家。

Project Summary Juvenile idiopathic arthritis-associated uveitis (JIA-U) is an inflammatory eye disease that affects 20% of children with JIA and can lead to cataracts, glaucoma, vision loss and blindness. Current screening guidelines are designed to identify children at risk for eye disease in JIA. However, pediatric rheumatologists' and ophthalmologists' ability to provide comprehensive screening for uveitis is limited because the guidelines have not been updated to include all juvenile arthritis subtypes and other known risk factors for vision-limiting complications of JIA. Screening in JIA and other related autoimmune diseases of childhood is crucial because early uveitis detection can prevent visual complications and influence therapeutic management. Likewise, institution of early aggressive therapy when needed may avert serious visual complications. To improve health outcomes and quality of life of children with JIA-U, we need a better understanding of risk factors for uveitis, long-term visual outcomes, and the effects of visual and physical disability on children's lives. Early and accurate identification of JIA-U through the identification of risk factors for susceptibility and severity of uveitis, standardization of outcome measures, and elucidation of the significance of HLA risk alleles would improve the evaluation of the impact of JIA-U and the understanding of disease etiology, pathogenesis and outcome. Likewise, an improved risk stratification scheme could reduce unnecessary ophthalmology visits and help us identify children at greater risk for severe eye disease and complications. The objectives of this study are to 1) Identify differences in the clinical characteristics and course of JIA-U in patients of European and African descent in children with JIA to identify clinical and genetic risk markers for uveitis, 2) Examine the risk factors identified over a 4-year prospective study of a cohort of children diagnosed with early JIA to determine which factors are associated with eventual development of uveitis, and provide data on their longitudinal profile, 3) Determine the impact of having both arthritis and uveitis on physical and visual disability, vision-specific QOL, and overall QOL using subjective and objective measures. Using a comprehensive biologic, genetic and psychosocial approach, we hope to develop more widely applicable risk stratification schemes based on JIA subtype, and to identify new risk factors that will identify the populations of children at greatest risk for severe disability and blindness so that interventions can be developed to improve their overall outcome. The candidate is a pediatric rheumatologist and an assistant professor of pediatrics at Emory University. She has obtained a Masters of Science in Clinical Investigation. She is devoted to a career in clinical outcomes research in children with autoimmune eye diseases. She has assembled an outstanding mentoring team with experience in pediatric rheumatology, pediatric ophthalmology, pediatric epidemiology, outcomes research and measurement, which are relevant to her objectives. This mentored award will help her become a successful independent clinician scientist.