佛兰德罕见结缔组织疾病 (CTD) 网络:系统性硬化症患者的病途. 采取了第一步,采取了第一步
Y Piette1,2,3, F Van den Bossche2, J Aerts4
1Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
Acta clinica Belgica
|December 18, 2023
概括
罕见病患者面临着重大的障碍. 这项研究概述了系统性硬化症 (SSc) 和其他罕见疾病的新患者途径,旨在通过专业网络提供公平,高质量的护理.
科学领域:
- 医学科学 医学科学 医学科学
- 罕见疾病 罕见疾病
- 医疗保健管理的管理
背景情况:
- 尽管个体患病率很低,但罕见疾病却造成了相当大的集体负担.
- 患者经常面临诊断延迟和有限的专家治疗机会.
- 欧洲参考网络 (ERN) 是为了通过促进跨境合作来应对这些挑战而建立的.
研究的目的:
- 为系统性硬化症 (SSc) 建立患者途径,作为罕见的连接和肌肉骨疾病的模型.
- 确保罕见病患者平等获得高质量的护理.
- 将ERN原则转化为区域医疗保健结构,以弗兰德罕见病网络为例.
主要方法:
- 为患者护理途径开发基于共识的关键信息.
- 定义SSc患者的多学科评估频率和设置.
- 确定复杂病例的监测和专业护理标准.
主要成果:
- 患有SSc的患者需要在三级中心进行基线和定期 (3-5年) 多学科评估.
- 在参考中心推每年进行临床评估,在遵循指南的中心允许进行技术评估.
- 专门的三级护理是早期扩散性皮肤性SSc,间歇性肺病和肺动脉高血压的强制性治疗.
结论:
- 建议的患者途径为系统性硬化症的管理提供了一个结构化的方法.
- 这些途径作为其他罕见的结合性和肌肉骨疾病的试点模型.
- 实施旨在改善区域罕见病网络中的护理质量和获得.
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