Developing Classification Criteria for the Uveitides

制定葡萄膜炎的分类标准

• 批准号: 9081760
• 负责人: Douglas A Jabs
• 金额: $ 42.86万
• 依托单位: ICAHN SCHOOL OF MEDICINE AT MOUNT SINAI
• 依托单位国家: 美国
• 财政年份: 2016
• 资助国家: 美国
• 起止时间: 2016-04-01 至 2020-03-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/9081760
• 关键词: Acute; Adult; Affect; Age; Agreement; Anatomy; Anterior uveitis; Antiviral Agents; Blindness; Child; Choroiditis; Chronic; Classification; Clinical Research; Clinical Trials; Collection; Complex; Consensus; Cytomegalovirus Retinitis; Data; Data Analyses; Databases; Development; Diabetic Retinopathy; Diagnosis; Diagnostic; Disease; Enrollment; Ensure; Epidemiology; Future; Genomics; Goals; Immune; Immunosuppression; Infection; Inflammation; Informatics; Intermediate Uveitis; Machine Learning; Mediating; Nomenclature; Outcomes Research; Panuveitis; Pathogenesis; Patients; Performance; Phase; Phenotype; Pigments; Population; Posterior Uveitis; Publications; Research; Retinitis; Risk Factors; Sensitivity and Specificity; Specificity; Standardization; Syndrome; Techniques; Terminology; Translational Research; United States; Uveitis; Vision; Visual impairment; Voting; Work; age group; age related; antimicrobial; birdshot chorioretinitis; cost; outcome forecast; public health relevance; symposium; tool; web page; working group

 DESCRIPTION (provided by applicant): The uveitides are a collection of ~30 distinct diseases characterized by intraocular infection. Each disease has its own features, course, treatment, and prognosis. Traditionally, the uveitides have been grouped by the primary anatomic site of inflammation as anterior uveitis, intermediate uveitis, posterior uveitis, and panuveitis. However, there are substantial limitations to this "lumping" of diseases. For example, among the posterior uveitides, some (e.g. toxoplasmic retinitis and cytomegalovirus retinitis) are infectious and require treatment with antimicrobial/antiviral agents, some are chronic, presumed immune-mediated diseases that require immunosuppression (e.g. birdshot chorioretinitis, multifocal choroiditis, serpiginous choroiditis), and a few are self-limited, spontaneously-remitting diseases with a good prognosis (e.g. acute posterior multifocal placoid pigment epitheliopathy and multiple evanescent white dot syndrome). As such precise diagnosis is critical for research, including epidemiology, translational pathogenesis research, outcomes research, and disease specific clinical trials. Classification criteria are a type of "diagnostic" criteria used for reserch purposes. Although classification criteria seek to optimize sensitivity and specificity, when a trade-off is required, they emphasize specificity in order to ensure that a homogeneous group of patients is being studied. A precise phenotype is required particularly for genomic risk factor studies of complex disorders and translational pathogenesis research, as inclusion of other diseases with different risk factors and disease mechanisms would confound the results. Currently there are no widely-accepted and validated classification criteria for any of the uveitides. Preliminary data indicate "fair to moderate" agreement at best on the independent diagnosis of any one case by uveitis experts (κ's 0.27-0.40), but the ability of committees to reach agreement on the diagnosis of >98% of cases. The goal of the "Developing Classification Criteria for the Uveitides" project is for the Standardization of Uveitis Nomenclature (SUN) Working Group to develop classification criteria for the 25 leading uveitides using a formal, rigorous approach. There are 4 phases to the project: 1) informatics, to develop a standardized terminology; 2) case collection, to develop a preliminary database of ~250 cases of each disease; 3) case selection, to select at least 150-200 cases of each disease that are generally accepted to be the disease (using formal consensus techniques) from the preliminary database into a final database; and 4) data analysis, using machine learning approaches, of the final database to develop a parsimonious set of criteria for each disease that minimizes misclassification. The informatics and case collection phases of the Project are complete. The case selection phase is well underway and uses online voting and consensus conference calls to achieve supermajority acceptance on all cases included in the final database. The goals of this application are to complete case selection and data analysis and develop classification criteria for the 25 of the major uveitides. These results are crucial to future clinical research i the field of uveitis.

 描述（由适用提供）：葡萄叶叶是一个〜30种不同疾病的集合，其特征是眼内感染。每种疾病都有自己的特征，病程，治疗和预后。传统上，葡萄叶剂是由炎症的主要解剖部位分组为前葡萄膜炎，中间葡萄膜炎，后葡萄膜炎和帕氏炎。然而， 这种疾病的“集团”存在很大的局限性。例如，在后葡萄叶酸盐中，某些（例如弓形虫性视网膜炎和巨细胞病毒视网膜炎）是感染性的，需要用抗菌/抗病毒剂治疗，有些是慢性，假定的，假定的免疫介导的疾病，这些疾病需要免疫介导的免疫抑制作用（例如，血清疗法（例如，五个鸟类），近距离鸟类炎，近距离鸟类炎，近距离晶体炎细封素，近乎近乎鸟类素蛋白素，是近距离刺激性的胰岛素性疾病，造成了较不利的晶体素素素素素，造成了较不足的鸟类素毒素，较为鸟胺素质素，较鸟类炎细布了胰岛素炎。脉络膜炎）和一些是自限的，赞助的疾病 具有良好的预后（例如，急性后验多焦点色素上皮病和多个evanevanscent的白点综合征）。由于这种精确的诊断对于研究至关重要，包括流行病学，转化发病机理研究，结果研究和特定于疾病的临床试验。分类标准是用于储备目的的一种“诊断”标准。尽管分类标准试图优化敏感性和特异性，但是当需要权衡取舍时，他们强调特异性，以确保研究一组同质的患者。对于复杂疾病和翻译发病机理研究的基因组危险因素研究，需要精确的表型，因为纳入具有不同危险因素和疾病机制的其他疾病会使结果混淆。目前，尚无对任何uveitides的广泛认可和验证的分类标准。初步数据表明，在葡萄膜炎专家（κ的0.27-0.40）对任何一种病例的独立诊断上，充其量是“公平至中等”的一致性，但委员会就诊断案件达成一致的能力> 98％。 “开发葡萄象体”项目的“开发分类标准”的目的是使用正式的，严格的方法来制定25个领先的葡萄叶叶替象的分类标准。该项目有4个阶段：1）信息，以开发标准化的术语； 2）案例收集，以开发约250例疾病病例的初步数据库； 3）案例选择，从初步数据库中选择至少150-200例的每种疾病病例，这些病例通常被认为是疾病（使用正式共识技术）； 4）使用机器学习方法的数据分析最终数据库为每种疾病制定一套简约的标准，以最大程度地减少错误分类。该项目的信息和案例收集阶段已完成。案例选择阶段正在进行中，并使用在线投票和共识电话会议，以在最终数据库中包含的所有案例中获得超大差的接受。该应用程序的目标是完成案例选择，数据分析和开发分类标准，该标准为25个主要的uveitides。这些结果对于未来的临床研究至关重要。