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嗜酸性肉芽肿性多血管炎(EGPA)的新视角:欧洲 EGPA 研究组第一次会议报告。

New perspectives in eosinophilic granulomatosis with polyangiitis (EGPA): report of the first meeting of the European EGPA Study Group.

机构信息

University of Modena and Reggio Emilia, and Azienda USL-IRCCS, Reggio Emilia, Italy.

Department of Medicine and Surgery, University of Milano-Bicocca and ASST Monza, Monza, Italy.

出版信息

Intern Emerg Med. 2019 Nov;14(8):1193-1197. doi: 10.1007/s11739-019-02166-5. Epub 2019 Aug 6.

Abstract

The European Eosinophilic Granulomatosis with Polyangiitis (EGPA) study group first gathered in Firenze in December 2018. The discussion was centred around the clinical and therapeutic needs in EGPA which still remain unmet. Indeed, EGPA is a puzzling and rare disease which shares clinical features with other anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAVs) and hypereosinophilic syndromes (HESs). Some of the recommendations published in 2015 are based on data derived from EGPA-related diseases, rather than from EGPA itself, and therefore need to be updated. Thus, the aim of the meeting was to stimulate ongoing research, to promote collaborative European studies and to define the main issues on which future studies should be focused. Current fields of research on EGPA include potential serological biomarkers of disease activity and of specific organ involvement, possible links between different genetic variants and clinical phenotypes, and new therapeutic perspectives. Herein, we give an overview of the meeting with the goal to stimulate an international collaboration and new points of discussion.

摘要

欧洲嗜酸性肉芽肿伴多血管炎(EGPA)研究组于 2018 年 12 月首次在佛罗伦萨聚集。讨论集中在 EGPA 仍未满足的临床和治疗需求上。事实上,EGPA 是一种令人困惑且罕见的疾病,其临床特征与其他抗中性粒细胞胞质抗体(ANCA)相关性血管炎(AAV)和高嗜酸性粒细胞综合征(HES)相似。2015 年发布的一些建议是基于源自 EGPA 相关疾病的数据,而不是源自 EGPA 本身,因此需要更新。因此,会议的目的是激发正在进行的研究,促进欧洲的合作研究,并确定未来研究应关注的主要问题。目前 EGPA 的研究领域包括疾病活动和特定器官受累的潜在血清学生物标志物、不同遗传变异与临床表型之间的可能联系,以及新的治疗前景。在此,我们概述了会议的内容,旨在激发国际合作和新的讨论点。

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