University of Manchester, Manchester Academic Health Science Centre, Salford Royal Hospital, Salford M6 8HD, UK.
Nat Rev Rheumatol. 2012 Aug;8(8):469-79. doi: 10.1038/nrrheum.2012.96. Epub 2012 Jul 10.
The past 10 years have seen the publication of results from several multicentre clinical trials in primary and systemic sclerosis (SSc)-related Raynaud phenomenon. The publication of these studies has occurred as a result of new insights into the pathogenesis of Raynaud phenomenon, which are directing new treatment approaches, and increased international collaboration between clinicians and scientists. Although the pathogenesis of Raynaud phenomenon is complex, abnormalities of the blood vessel wall, of neural control mechanisms and of intravascular (circulating) factors are known to interact and contribute. Key players relevant in drug development include nitric oxide, endothelin-1, alpha adrenergic receptor activation, abnormal signal transduction in vascular smooth muscle, oxidative stress and platelet activation. The main advances in diagnosis have been a clearer understanding of autoantibodies and of abnormal nailfold capillary patterns as independent predictors of SSc, and widespread use and increased availability of capillaroscopy. The ultimate aim is to translate the advances made in the pathophysiology and early diagnosis into development of treatments to prevent and reverse digital vascular dysfunction and injury. This Review provides an update of the pathogenesis, diagnosis and treatment of Raynaud phenomenon. Current and future treatment approaches are discussed, and some key unanswered questions are highlighted.
过去 10 年中，已经有几项关于原发性和系统性硬化症（SSc）相关雷诺现象的多中心临床试验结果发表。这些研究的发表是由于对雷诺现象发病机制的新认识，这些认识指导了新的治疗方法，并促进了临床医生和科学家之间的国际合作。尽管雷诺现象的发病机制很复杂，但已知血管壁、神经控制机制和血管内（循环）因子的异常相互作用并起作用。药物开发中的关键参与者包括一氧化氮、内皮素-1、α肾上腺素能受体激活、血管平滑肌异常信号转导、氧化应激和血小板激活。诊断方面的主要进展是更清楚地了解自身抗体和异常甲襞毛细血管模式作为 SSc 的独立预测因素，以及毛细血管镜的广泛使用和可用性增加。最终目标是将病理生理学和早期诊断方面的进展转化为预防和逆转数字血管功能障碍和损伤的治疗方法。本综述介绍了雷诺现象的发病机制、诊断和治疗的最新进展。讨论了当前和未来的治疗方法，并强调了一些关键的未解决问题。
