Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.
Department of Medicine, Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, USA.
Best Pract Res Clin Rheumatol. 2020 Feb;34(1):101474. doi: 10.1016/j.berh.2019.101474. Epub 2020 Jan 29.
Raynaud's phenomenon (RP) is common, affecting approximately 5% of the population, and is important to the rheumatologist because it is often the presenting symptom of connective tissue disease, especially of systemic sclerosis (SSc)-spectrum disorders. RP therefore provides a window of opportunity for early diagnosis. When RP is associated with SSc it is particularly challenging to treat. This review begins with a discussion of some of the recent advances in our understanding of the pathogenesis of RP: it is through increased understanding of the complex pathophysiology of RP that we are most likely to develop new therapies. The following questions are then addressed (with three clinical scenarios demonstrating key principles of assessment and management): 1. How can we predict underlying connective tissue disease in the patient presenting with Raynaud's? 2. How can we measure severity of Raynaud's? 3. What are the latest advances in treatment of connective tissue disease-related digital vasculopathy?
雷诺现象（RP）很常见，大约影响 5%的人群，对于风湿病学家很重要，因为它常常是结缔组织病的首发症状，尤其是系统性硬化症（SSc）谱系疾病。因此，RP 为早期诊断提供了机会。当 RP 与 SSc 相关时，治疗尤其具有挑战性。本综述首先讨论了我们对 RP 发病机制的一些最新认识方面的进展：正是通过对 RP 复杂病理生理学的深入了解，我们最有可能开发出新的治疗方法。然后讨论了以下问题（通过三个临床病例展示评估和管理的关键原则）：1. 如何在出现雷诺现象的患者中预测潜在的结缔组织病？2. 如何衡量雷诺现象的严重程度？3. 结缔组织病相关数字血管病变治疗的最新进展是什么？
