Bajema Ingeborg M, Bruijn Jan A, Casian Alina, Cid Maria C, Csernok Elena, van Daalen Emma, Harper Lorraine, Hauser Thomas, Little Mark A, Luqmani Raashid A, Mahr Alfred, Ponte Cristina, Salama Alan, Segelmark Mårten, Suzuki Kazuo, Sznajd Jan, Teng Y K Onno, Vaglio Augusto, Westman Kerstin, Jayne David
Department of Pathology, Leiden University Medical Center, Leiden, the Netherlands.
Guy's Hospital, London, UK.
Kidney Int Rep. 2017 Sep 21;2(6):1018-1031. doi: 10.1016/j.ekir.2017.09.008. eCollection 2017 Nov.
The 2016 European Vasculitis Society (EUVAS) meeting, held in Leiden, the Netherlands, was centered around phenotypic subtyping in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). There were parallel meetings of the EUVAS petals, which here report on disease assessment; database; and long-term follow-up, registries, genetics, histology, biomarker studies, and clinical trials. Studies currently conducted will improve our ability to discriminate between different forms of vasculitis. In a project that involves the 10-year follow-up of AAV patients, we are working on retrieving data on patient and renal survival, relapse rate, the cumulative incidence of malignancies, and comorbidities. Across Europe, several vasculitis registries were developed covering over 10,000 registered patients. In the near future, these registries will facilitate clinical research in AAV on a scale hitherto unknown. Current studies on the genetic background of AAV will explore the potential prognostic significance of genetic markers and further refine genetic associations with distinct disease subsets. The histopathological classification of ANCA-associated glomerulonephritis is currently evaluated in light of data coming out of a large international validation study. In our continuous search for biomarkers to predict clinical outcome, promising new markers are important subjects of current research. Over the last 2 decades, a host of clinical trials have provided evidence for refinement of therapeutic regimens. We give an overview of clinical trials currently under development, and consider refractory vasculitis in detail. The goal of EUVAS is to stimulate ongoing research in clinical, serological, and histological management and techniques for patients with systemic vasculitis, with an outlook on the applicability for clinical trials.
2016年欧洲血管炎学会（EUVAS）会议在荷兰莱顿举行，会议主要围绕抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）的表型分型展开。EUVAS各专项会议同期举行，本文汇报了疾病评估、数据库、长期随访、登记注册、遗传学、组织学、生物标志物研究及临床试验等方面的情况。目前开展的研究将提高我们鉴别不同形式血管炎的能力。在一个涉及AAV患者10年随访的项目中，我们正致力于获取有关患者生存率、肾脏生存率、复发率、恶性肿瘤累积发病率及合并症的数据。在欧洲各地，已建立了多个血管炎登记处，登记患者超过10000例。在不久的将来，这些登记处将推动AAV领域前所未有的大规模临床研究。目前关于AAV遗传背景的研究将探索遗传标志物的潜在预后意义，并进一步完善与不同疾病亚组的遗传关联。目前正在根据一项大型国际验证研究的数据，对ANCA相关肾小球肾炎的组织病理学分类进行评估。在我们持续寻找预测临床结局的生物标志物的过程中，有前景的新标志物是当前研究的重要课题。在过去20年里，大量临床试验为优化治疗方案提供了证据。我们概述了目前正在开展的临床试验，并详细探讨了难治性血管炎。EUVAS的目标是推动针对系统性血管炎患者的临床、血清学及组织学管理和技术方面的现有研究，并展望其在临床试验中的适用性。
