Raynaud phenomenon and digital ulcers in systemic sclerosis.,Nature Reviews Rheumatology

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Raynaud phenomenon and digital ulcers in systemic sclerosis.
Nature Reviews Rheumatology ( IF 29.4 ) Pub Date : 2020-02-25 , DOI: 10.1038/s41584-020-0386-4
Michael Hughes _{1,

2} , Yannick Allanore ₃ , Lorinda Chung ₄ , John D Pauling _{5,

6} , Christopher P Denton ₇ , Marco Matucci-Cerinic ₈

Affiliation

Raynaud phenomenon is a symptom complex caused by impaired digital perfusion and can occur as a primary phenomenon or secondary to a wide range of underlying causes. Raynaud phenomenon occurs in virtually all patients with systemic sclerosis (SSc) and is often the earliest clinical manifestation to occur. Careful assessment is required in patients with Raynaud phenomenon to avoid missing secondary causes such as SSc. Digital ulcers are a painful and disabling visible manifestation of digital vascular injury in patients with SSc. Progress has been made in the classification and assessment of digital ulcers and in understanding ulcer pathogenesis, and there are a wide range of treatments available to both prevent and heal digital ulcers, some of which are also used in Raynaud phenomenon management. In this Review, the assessment of patients with Raynaud phenomenon is discussed, including 'red flags' that are suggestive of SSc. The pathogenesis, classification and assessment of SSc-associated digital ulcers are also covered, alongside an overview of management approaches for SSc-associated Raynaud phenomenon and digital ulcers. Finally, unmet needs are discussed and the concept of a unified vascular phenotype in which therapies that affect the vasculature to support disease modification strategies is introduced.

中文翻译：

雷诺现象和系统性硬化症中的数字溃疡。

雷诺现象是由数字灌注不足引起的症状复合体，可以作为主要现象发生，也可以作为许多潜在原因的次要现象发生。几乎所有的系统性硬化症（SSc）患者都出现雷诺现象，并且通常是最早的临床表现。有雷诺现象的患者需要仔细评估，以避免遗漏诸如SSc的次要原因。在SSc患者中，数字溃疡是数字血管损伤的一种痛苦且致残的可见表现。在数字溃疡的分类和评估以及对溃疡发病机理的理解方面已经取得了进展，并且有各种各样的预防和治愈数字溃疡的治疗方法，其中一些也用于雷诺现象管理中。在这篇评论中，讨论了雷诺现象患者的评估，包括提示SSc的“红色标志”。还介绍了与SSc相关的数字溃疡的发病机理，分类和评估，以及与SSc相关的Raynaud现象和数字溃疡的管理方法的概述。最后，讨论了未满足的需求，并介绍了统一血管表型的概念，其中介绍了影响脉管系统以支持疾病改良策略的疗法。

更新日期：2020-02-25

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