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雷诺现象

Fingers that go white, blue then red in the cold — primary vs secondary Raynaud's, keeping warm, and when it needs assessment.

一只张开的手,两个指尖呈苍白色,一个呈蓝色,一个呈潮红色——雷诺现象发作的三种颜色阶段。
在雷诺现象发作时,受累手指因小血管收缩而变得苍白和冰冷,随后在血液回流时变为蓝色,继而转为红色。 Kieran Hirpara 4.0

本页面由机器翻译,尚未经临床医生审核。英文版本为权威版本。

您的感受

当手指受冷或处于压力状态时,会出现颜色改变和异常感觉。 典型的发作过程分为三个阶段:手指首先变为白色,感觉冰冷且麻木;随后随着血液流失而变为蓝色;最后随着血液重新涌入而变为红色,并可能伴有搏动或刺痛感。在此过程中,您可能会注意到麻木、针刺感或酸痛。同样的情况有时也会影响脚趾、鼻子、耳朵或嘴唇。

发作时好时坏。它们通常由微小的诱因触发(如从冷冻室取出食物、寒冷的早晨、手持冰镇饮料或突然的压力波),一旦身体回暖,通常在几分钟到半小时内自行缓解。在发作间歇期,手指的外观和感觉通常完全正常。

实际发生了什么

为手指供血的小血管在寒冷环境中会收缩,以维持核心体温。在雷诺现象中,这些血管反应过度,收缩程度远超所需。这被称为血管痉挛。在短暂的时间内,极少量的血液到达手指皮肤:这就是苍白、冰冷、麻木的阶段。随着痉挛缓解,血液回流,手指变为蓝色,随后又变红并恢复温暖。

了解其两种类型很有帮助。原发性雷诺现象是最常见的类型,本质上仅是身体对寒冷过度敏感,背后并无潜在疾病。它常始于年轻女性,双手受累较为对称,尽管可能带来不便,但通常无害。继发性雷诺现象较少见但更为重要,因为此处的血管痉挛与另一种疾病相关,通常是影响结缔组织的疾病,如硬皮病或狼疮。指向继发性类型的线索包括:发作在较晚年龄才开始;发作累及单侧手或仅少数几根手指,而非双手对称受累;发作非常严重;指尖出现疮口或溃疡;或出现关节疼痛或皮疹等其他症状。这些特征值得进行正规的医学检查。

我们可以采取的措施

对大多数人而言,主要方法简单且有效:保持温暖并避免诱发因素。

  • 提前应对寒冷。 戴手套(连指手套能让手指并拢,从而更保暖),使用暖手宝,并分层穿衣。保持全身及核心体温与手部保暖同样重要;当核心体温适宜时,手指血管会保持开放。
  • 规避突然的寒冷。 戴手套去拿冰箱或冷冻室里的物品,提前开启汽车暖风,并将冰冷的罐装或瓶装饮料放在隔热杯套中握持。
  • 戒烟。 吸烟会使血管收缩,加重雷诺现象,戒烟是您能做的最有用的事情之一。
  • 减少咖啡因摄入,并避免任何会让您兴奋的物质,因为两者都可能诱发发作。采用适合您的方式管理压力也有帮助,因为压力本身即可诱发血管痉挛。

如果发作频繁、疼痛或严重,有药物可以放松血管并减少发作频率。最常见的一类是钙通道阻滞剂(如硝苯地平),如果这类药物不适合,还有其他选择。如果病因是继发性雷诺现象,治疗基础疾病是计划中的重要部分,因此获得正确的诊断至关重要。

预期情况

对于绝大多数患者(原发性雷诺现象者)而言,这更多是一种可管理的困扰,而非危险。通过保持适度保暖并避免诱发因素,许多人能将发作降至最低限度并正常生活;每次发作后手指均可完全恢复,不会造成永久性损害。这通常是一种长期倾向,而非会自行消失的情况,但非常可控,当简单措施不足以控制症状时,药物可作为补充手段。

继发性雷诺现象需要更密切的关注,因为基础疾病决定了病情走向,且在某些情况下,血流减少可能损伤指尖皮肤。这正是明确雷诺现象类型的重要性所在,以便为您的具体情况匹配适当级别的护理。

何时就医

如出现以下情况,请就医评估:

  • 您的发作始于成年后期、症状严重,或仅影响一只手或少数几根手指,而非双手均匀受累:这些可能是继发型雷诺现象的征兆,通常需要进行血液检查并检查指甲根部的小血管。
  • 您的指尖出现疮口、溃疡、裂口或皮肤破损,或手指某区域持续发白、发蓝、疼痛或麻木且无法复温:这需要及时关注。
  • 除颜色变化外,您还伴有其他症状:关节疼痛或肿胀、皮疹、眼干或口干、吞咽困难,或手指皮肤紧绷或增厚。
  • 尽管注意保暖并避免诱发因素,发作仍频繁或疼痛到足以干扰您的日常生活;药物可能有所帮助。

深入探讨

Advanced reading: the deeper science (optional)

本节内容超出了您自身治疗决策所需的范围。雷诺现象值得额外阅读,因为只有一类药物拥有良好的证据支持,且当前最受关注的疗法通过一种出乎意料的机制发挥作用。

有一类药物具有循证依据

雷诺现象是手指小动脉的阵发性痉挛,因此治疗的目标是保持这些血管的开放。许多血管扩张剂已被尝试使用。

一项针对17项试验的荟萃分析得出结论:钙通道阻滞剂在减少症状的频率和严重程度方面显示出具有统计学意义的益处,并且没有证据支持钙通道阻滞剂以外的口服血管扩张剂[1]。

第二个分句是实用信息。它是对整个替代药物类别的阴性发现,并解释了为何治疗往往从一个药物家族开始,并且常常一直使用它,而不是逐一尝试一系列药物。

肉毒毒素,以及它为何并非如你所猜测的那样起效

在难治性病例中,引发最多关注的干预措施是在手指基部的动脉周围注射肉毒毒素。其用于对药物治疗无反应的雷诺现象已显示出良好结果,作为一种在小型研究中已证实有效的手术非侵入性替代方案 [2]。

其机制值得理解,因为它是反直觉的。肉毒毒素以麻痹肌肉而闻名,显而易见的猜测是它直接松弛动脉壁中的肌肉。目前的理解有所不同:它干扰驱动血管收缩的交感神经信号,并且似乎还作用于疼痛通路,这就是为什么报告的益处有时包括与血流变化不成比例的疼痛缓解。

请注意证据本身的诚实表述:“小型研究”。对于已穷尽药物治疗的人群而言,这是一个有前景的选择,而非一个已确立的选择。

决定其他一切的关键区别

最具决定性的问题是,该现象是原发性的,即独立发生,还是继发性的,由潜在疾病引起,最常见的是自身免疫性结缔组织病,如硬皮病或狼疮。

这一区别并非纯理论问题。原发性雷诺现象是一种预后良好且无组织损伤的困扰。继发性雷诺现象可能进展至指尖溃疡和组织缺损,且潜在疾病本身需要被识别和治疗。

引起警惕的特征包括:发病年龄约在30岁之后、双手不对称、指尖出现溃疡或凹陷性瘢痕,以及甲襞毛细血管异常。这些情况需要进行检查而非仅仅给予安慰,这也是雷诺现象的首次就诊通常需要进行全面评估,而不仅仅是通过戴手套进行管理的原因。

手术在阶梯中的位置

对于伴有组织受威胁的严重继发性疾病,动脉周围交感神经切除术——即剥离指动脉表面的交感神经纤维——是手术选择,它通过永久性途径靶向与肉毒毒素相同的信号通路。该手术仅保留用于严重缺血和不愈合的溃疡,而非用于手指发冷、不适,这与上述治疗阶梯一致:阶梯推进得越远,目标就越从舒适转向挽救组织。

参考文献

[1] Butendieck RR, Murray PM. Raynaud disease. J Hand Surg Am. 2014;39(1):121-4. https://doi.org/10.1016/j.jhsa.2013.08.117

[2] Gallegos JE, Inglesby DC, Young ZT, Herrera FA. Botulinum toxin for the treatment of intractable Raynaud phenomenon. J Hand Surg Am. 2021;46(1):54-9. https://doi.org/10.1016/j.jhsa.2020.07.009


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