Abstract

Introduction

The rarity of systemic sclerosis (SSc) and its widely heterogeneous presentation and disease course are the main limitations for clinical research. The European Scleroderma Trials and Research group (EUSTAR) was launched in 2004, aiming to unify research efforts in the field of SSc. The central EUSTAR database has grown exponentially over the years, promoting new research and clinical trials, shedding new light on SSc diagnosis, its clinical course and providing new ideas for state-of-the-art therapy.Areas covered: The authors summarized key findings of the main EUSTAR studies by reviewing PubMed and Web-of-Science databases through July 2020. The authors focused on the very early diagnosis of SSc, the prediction of disease course and mortality, the evaluation of disease activity and quality of life, the general management and therapy.Expert opinion: The findings elucidated in EUSTAR studies have substantially improved the diagnostic and therapeutic approach to SSc in the last fifteen years. Further efforts are warranted to identify early prognostic markers of the disease and stratify patients who may benefit most from vasoactive, immunosuppressive, and/or antifibrotic therapy. This will be particularly important in leading the future of SSc towards precision medicine and to promote more targeted clinical trials.

中文翻译：

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EUSTAR 队列的力量：迄今为止的主要发现及其对系统性硬化症患者管理的影响

摘要

介绍

系统性硬化症 (SSc) 的罕见性及其广泛的异质性表现和病程是临床研究的主要限制。欧洲硬皮病试验和研究小组 (EUSTAR) 于 2004 年成立，旨在统一 SSc 领域的研究工作。多年来，EUSTAR 中央数据库呈指数级增长，促进了新的研究和临床试验，为 SSc 诊断及其临床过程提供了新的视角，并为最先进的治疗提供了新思路。覆盖区域：作者通过审查截至 2020 年 7 月的 PubMed 和科学网络数据库，总结了主要 EUSTAR 研究的主要发现。作者重点关注 SSc 的极早期诊断、病程和死亡率的预测、疾病活动性的评估和生活质量，一般管理和治疗。专家意见：EUSTAR 研究中阐明的发现在过去十五年中大大改进了 SSc 的诊断和治疗方法。需要进一步努力来确定疾病的早期预后标志物，并对可能从血管活性、免疫抑制和/或抗纤维化治疗中获益最多的患者进行分层。这对于引领 SSc 的未来走向精准医学和促进更有针对性的临床试验尤为重要。