Outcomes of children with juvenile idiopathic arthritis-associated uveitis

幼年特发性关节炎相关葡萄膜炎儿童的结局

基本信息

批准号：
8310928
负责人：
Sheila Therese Angeles-Han
金额：
$ 19.6万
依托单位：
EMORY UNIVERSITY
依托单位国家：
美国
项目类别：
财政年份：
2011
资助国家：
美国
起止时间：
2011-09-01 至 2016-08-31
项目状态：
已结题

来源：
https://reporter.nih.gov/project-details/8310928
关键词：
Academy Affect African African American Age Algorithms Alleles American Antinuclear Antibodies Arthritis Autoimmune Diseases Autoimmune Process Award Blindness Cataract Cells Characteristics Child Childhood Chronic Childhood Arthritis Chronic Disease Clinical Clinical Trials Cohort Studies Complement Complication Data Databases Detection Development Diagnosis Disease Early Diagnosis Epidemiology Etiology European Evaluation Eye Eye diseases Flare Genetic Genetic Polymorphism Genetic Risk Glaucoma Goals Guidelines HLA-B27 Antigen HLA-DR1 Antigen Health Healthcare Impact evaluation Impairment Inflammatory Institution Intervention Lead Master of Science Measurement Measures Medical Records Mentors Monitor Onset of illness Ophthalmologist Ophthalmology Outcome Outcome Measure Outcomes Research Pathogenesis Patient-Focused Outcomes Patients Pediatrics Play Population Predisposition Prospective Studies Proteins Psyche structure Quality of life Questionnaires Race Research Resources Rheumatism Rheumatology Risk Risk Factors Risk Marker Role Scheme Scientist Screening procedure Secondary to Severities Severity of illness Social Functioning Standardization Stratification Symptoms Therapeutic United States Universities Update Uveitis Variant Vision Vision Disorders Visit Visual Visual Acuity Visual impairment aggressive therapy base career clinical epidemiology cohort design disability disorder risk experience genetic risk factor global health illness length improved longitudinal course prevent professor prospective psychologic psychosocial rheumatologist tertiary care tool

项目摘要

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.

项目概要幼年特发性关节炎相关葡萄膜炎 (JIA-U) 是一种炎症性眼病，影响 20% 的眼部疾病。患有幼年特发性关节炎的儿童可能会导致白内障、青光眼、视力丧失和失明。目前的筛查指南旨在识别有幼年特发性眼病风险的儿童。然而，儿科风湿病学家和眼科医生提供葡萄膜炎全面筛查的能力有限，因为指南已尚未更新以包括所有幼年关节炎亚型和其他已知的视力限制风险因素 JIA 的并发症。幼年特发性关节炎和其他相关儿童自身免疫性疾病的筛查至关重要，因为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，必要时进行早期积极治疗可能会避免严重的视力并发症。改善健康 JIA-U 儿童的结局和生活质量，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。早期和通过识别葡萄膜炎易感性和严重程度的危险因素来准确识别 JIA-U，结果测量的标准化以及 HLA 风险等位基因重要性的阐明将改善评估 JIA-U 的影响以及了解疾病的病因、发病机制和结果。同样，改进的风险分层方案可以减少不必要的眼科就诊并帮助我们识别患有严重眼病和并发症的较高风险的儿童。本研究的目的是 1) 确定 JIA-U 临床特征和病程的差异欧洲和非洲血统的幼年特发性关节炎儿童患者，以确定临床和遗传风险标记葡萄膜炎，2) 检查对一组诊断为葡萄膜炎的儿童进行的为期 4 年的前瞻性研究中确定的危险因素与早期 JIA 一起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使用主观和客观测量的残疾、特定视力的生活质量和总体生活质量。使用综合生物学、遗传学和心理社会方法，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，从而识别 JIA 人群儿童患有严重残疾和失明的风险最大，因此可以制定干预措施来改善他们的总体结果。该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患有自身免疫性眼病的儿童的研究。她组建了一支优秀的导师团队在儿科风湿病学、儿科眼科、儿科流行病学、结果研究和测量，这与她的目标相关。这个指导奖项将帮助她成为一名成功的独立临床科学家。