Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, Botnar Research Centre, Nuffield Orthopaedic Centre, University of Oxford, Windmill Road, Oxford, OX3 7LD, UK.
Rheumatology and Metabolic Bone Diseases Department, Hospital de Santa Maria, CHLN, Lisbon, Portugal.
Clin Exp Nephrol. 2013 Oct;17(5):619-621. doi: 10.1007/s10157-013-0854-0. Epub 2013 Aug 31.
The systemic vasculitides are a group of uncommon diseases characterized by blood vessel inflammation. There are currently no diagnostic criteria for the primary systemic vasculitides and physicians must rely on experience and disease definitions. The absence of validated criteria can result in delays in making the correct diagnosis and starting appropriate therapy. With the increased understanding of the pathophysiology of vasculitis and newer diagnostic tests in widespread clinical use, it is an appropriate time for classification criteria for primary vasculitis to be revised. The Diagnostic and Classification Criteria for Vasculitis (DCVAS) study is a multinational observational study designed to develop and validate diagnostic criteria and to improve and validate classification criteria for primary systemic vasculitis. The analytic approach will be based on the traditional approach of vessel size for classification of vasculitis but will also incorporate detailed clinical data, evaluation of anti-neutrophil cytoplasm antibody diagnostic testing, biopsy and imaging data. The study is following the guidelines for the development of classification criteria established by the American College of Rheumatology and the European League against Rheumatism. The study will incorporate the use of pre-defined cases of each condition to reduce the inherent circularity when developing new classification criteria and will explore alternative approaches to deriving reference standards by creating data-driven classification algorithms. We anticipate recruiting >2,000 patients with primary systemic vasculitis and 1,500 patients with autoimmune diseases and other conditions that mimic vasculitis. As of June 2013, >100 medical centers across 31 countries in Asia, Australasia, Europe, North America, and South America were contributing data to the study. The DCVAS study provides a unique opportunity to increase generalizability and collate a large dataset on the occurrence, presentation, and outcome of vasculitis in different populations.
系统性血管炎是一组以血管炎症为特征的罕见疾病。目前尚无原发性系统性血管炎的诊断标准，医生必须依靠经验和疾病定义。缺乏经过验证的标准可能导致正确诊断和开始适当治疗的延迟。随着对血管炎病理生理学的理解的增加和新的诊断测试在广泛的临床应用，现在是修订原发性血管炎分类标准的适当时机。血管炎的诊断和分类标准（DCVAS）研究是一项多中心观察性研究，旨在制定和验证诊断标准，并改进和验证原发性系统性血管炎的分类标准。分析方法将基于血管大小的传统分类方法，但也将纳入详细的临床数据、抗中性粒细胞胞浆抗体诊断检测的评估、活检和影像学数据。该研究遵循美国风湿病学会和欧洲抗风湿病联盟制定的分类标准制定指南。该研究将纳入每种疾病的预定义病例，以减少在制定新分类标准时固有的循环性，并通过创建基于数据的分类算法探索得出参考标准的替代方法。我们预计将招募>2000 例原发性系统性血管炎患者和 1500 例自身免疫性疾病和其他模拟血管炎的患者。截至 2013 年 6 月，来自亚洲、澳大拉西亚、欧洲、北美和南美 31 个国家的 100 多个医疗中心正在向该研究提供数据。DCVAS 研究提供了一个独特的机会，可以增加普遍性并整理不同人群中血管炎发生、表现和结果的大型数据集。
