Riemekasten Gabriela, Müller-Ladner Ulf
Klinik für Rheumatologie und Klinische Immunologie, Universitätsklinikum Schleswig-Holstein Campus Lübeck, Forschungszentrum Borstel, Universität zu Lübeck, Ratzeburger Allee 160, 23538, Lübeck Deutschland, Deutschland.
Abteilung für Rheumatologie und Klinische Immunologie, Campus Kerckhoff, Justus-Liebig Universität Gießen, Bad Nauheim, Deutschland.
Z Rheumatol. 2024 Aug;83(6):460-470. doi: 10.1007/s00393-024-01552-5. Epub 2024 Aug 2.
The updates to the European recommendations and the German guidelines for the treatment of systemic sclerosis are expected shortly, which are very good evidence-based guidelines for all those treating the disease; however, there are still disease manifestations with insufficient studies and current study results that were published after the review of the literature for the guidelines and might be of interest to the reader.
The aim of this work is to provide an overview of the publications in the last year that are interesting from the authors' point of view. The aim is to provide practically relevant information on the current state of knowledge that can supplement the guidelines.
The pathogenesis of systemic sclerosis (SSc) is becoming better understood in its interplay between environmental factors and the development of autoantibodies. There have also been overviews of the manifestation and prognosis of cardiac involvement in the last year. The American Thoracic Society issued the first guidelines for the treatment of interstitial lung disease in SSc. There are an increasing number of studies that suggest that disease-modulating combination therapies, such as rituximab and mycophenolate mofetil (MMF) are beneficial. Work addressing the involvement of joints suggests that inflammatory changes are common. Current options for the treatment of gastrointestinal involvement are presented.
The diagnosis and treatment of systemic sclerosis is making progress and many symptoms and complications are treatable. Nevertheless, much remains to be done to improve the quality of life of the patients.
欧洲系统性硬化症治疗建议及德国相关指南的更新预计不久后发布，这些指南为所有治疗该疾病的人员提供了非常好的循证依据；然而，仍有一些疾病表现的研究不足，且当前的研究结果是在指南文献综述之后发表的，可能会引起读者的兴趣。
这项工作的目的是概述作者认为过去一年中有意思的出版物。目的是提供与当前知识状态相关的实用信息，以补充指南内容。
系统性硬化症（SSc）的发病机制在环境因素与自身抗体形成之间的相互作用方面正逐渐被更好地理解。去年也有关于心脏受累表现及预后的综述。美国胸科学会发布了首个系统性硬化症间质性肺疾病治疗指南。越来越多的研究表明，诸如利妥昔单抗和霉酚酸酯（MMF）等疾病调节联合疗法是有益的。有关关节受累的研究表明炎症改变很常见。介绍了目前治疗胃肠道受累的方法。
系统性硬化症的诊断和治疗正在取得进展，许多症状和并发症是可治疗的。然而，要改善患者的生活质量仍有许多工作要做。
