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

• 批准号: 8523893
• 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
• 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/8523893
• 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; 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; clinical risk; cohort; design; disability; disorder risk; experience; genetic risk factor; global health; illness length; improved; longitudinal course; prevent; professor; prospective; psychologic; psychosocial; rheumatologist; screening; tertiary care; tool

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.

项目摘要 少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％ 贾亚（Jia）的孩子可以导致白内障，青光眼，视力丧失和失明。当前的筛选指南 旨在识别JIA患眼病风险的儿童。但是，小儿风湿病学家和 眼科医生提供葡萄膜炎的全面筛查的能力有限，因为这些准则具有 没有更新以包括所有少年关节炎亚型和其他已知风险因素的视力限制因素 贾的并发症。在JIA和其他相关的自身免疫性疾病中筛查儿童时期至关重要，因为 早期的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， 需要在需要时进行早期侵略性治疗的机构，可能会避免严重的视觉并发症。改善健康 JIA-U儿童的成果和生活质量，我们需要更好地了解葡萄膜炎的危险因素， 长期视觉结果以及视觉和身体残疾对儿童生活的影响。早 通过确定葡萄膜炎的易感性和严重程度的危险因素来准确识别JIA-U， 结果度量的标准化，并阐明HLA风险等位基因的意义将改善 评估JIA-U的影响以及对疾病病因，发病机理和结果的理解。 同样，改善的风险分层计划可以减少不必要的眼科访问并帮助我们 确定患有严重眼部疾病和并发症风险更大的儿童。 这项研究的目标是1）确定JIA-U的临床特征和过程的差异 JIA儿童的欧洲和非洲血统患者，以识别临床和遗传风险标志物的 葡萄膜炎，2）检查在一组被诊断的儿童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有关，并提供数据 关于其纵向剖面，3）确定患有关节炎和葡萄膜炎对身体和视觉的影响 使用主观和客观措施，残疾，特定的QOL和整体QOL。使用 全面的生物学，遗传和社会心理方法，我们希望发展更广泛的适用风险 基于JIA亚型的分层方案，并确定将确定种群的新风险因素 严重残疾和失明风险最大的孩子，以便开发干预措施以改善 他们的总体结果。 候选人是儿科风湿病学家和埃默里大学（Emory University）的儿科助理教授。她 已经获得了临床研究的科学硕士学位。她致力于临床成果的职业 对自身免疫性眼部疾病的儿童进行研究。她已经组建了一支杰出的指导团队 在小儿风湿病学，小儿眼科，小儿流行病学，成果研究和研究方面的经验 测量，与她的目标有关。这个指导的奖项将有助于她成为成功 独立的临床医生。