Klein-Weigel Peter, Sander Oliver, Reinhold Simone, Nielitz Jessica, Steindl Julia, Richter Jutta
Dtsch Arztebl Int. 2021 Apr 9;118(Forthcoming):273-80. doi: 10.3238/arztebl.m2021.0023.
Some 5-10% of the German population are affected by Raynaud's phenomenon (RP). In around 10-20% of cases RP arises from an underlying disease, most commonly a connective tissue disease. This review encompasses the diagnosis and differential diagnosis of RP and examines the efficacy of the currently available pharmaceutical and non-pharmaceutical treatment options.
We conducted a selective literature search in PubMed using the search terms "Raynaud's phenomenon", "Raynaud's syndrome," "vasospasm," "vascular acrosyndrome," and "systemic sclerosis," together with a search of the Cochrane Database of Systematic Reviews up to April 2020.
Raynaud's phenomenon mainly affects the fingers or toes and is typically triggered by cold or emotional stressors. The most important diagnostic steps are demonstration of a tendency towards digital vasospasm, exclusion of occlusions in the afferent arteries and acral vessels, nail-fold capillaroscopy, and determination of autoantibody status. Tumor screening should be arranged in the presence of B symptoms or first manifestation of RP in old age. The onset of RP in childhood is a rare occurrence and points to a secondary origin. The principal options for treatment are protection against cold and administration of calcium antagonists, which reduces the occurrence of RP by around 20-40 %. The treatment of RP in patients with systemic sclerosis is described in the recommendations of the European League Against Rheumatism (EULAR).
At onset or after years of latency, patients with Raynaud phenomenon may have an underlying disease (most commonly a connective tissue disease). Long-term specialist care is necessary for asymptomatic patients with risk factors and for those with clinically manifest symptoms of an underlying condition alike.
约5%-10%的德国人口受雷诺现象(RP)影响。在约10%-20%的病例中,RP由潜在疾病引起,最常见的是结缔组织病。本综述涵盖RP的诊断和鉴别诊断,并考察当前可用的药物和非药物治疗方案的疗效。
我们在PubMed中使用搜索词“雷诺现象”“雷诺综合征”“血管痉挛”“血管性肢端综合征”和“系统性硬化症”进行了选择性文献检索,并检索了截至2020年4月的Cochrane系统评价数据库。
雷诺现象主要影响手指或脚趾,通常由寒冷或情绪应激因素触发。最重要的诊断步骤是证明存在手指血管痉挛倾向、排除传入动脉和肢端血管阻塞、甲襞毛细血管镜检查以及确定自身抗体状态。在出现B症状或老年首次出现RP时应安排肿瘤筛查。儿童期出现RP很少见,提示为继发性病因。主要治疗选择是防寒和给予钙拮抗剂,这可使RP的发生率降低约20%-40%。欧洲抗风湿病联盟(EULAR)的建议中描述了系统性硬化症患者RP的治疗方法。
在发病时或数年潜伏期后,雷诺现象患者可能存在潜在疾病(最常见的是结缔组织病)。对于有危险因素的无症状患者以及有潜在疾病临床表现的患者,长期专科护理都是必要的。