Ture Hirut Yadeta, Lee Nan Young, Kim Na Ri, Nam Eon Jeong
Division of Rheumatology, Department of Internal Medicine, School of Medicine, Kyungpook National University, Daegu, Korea.
Department of Clinical Pathology, School of Medicine, Kyungpook National University, Daegu, Korea.
Vasc Specialist Int. 2024 Jul 23;40:26. doi: 10.5758/vsi.240047.
Raynaud's phenomenon (RP) is a condition characterized by episodic, excessive vasoconstriction in the fingers and toes, triggered by cold or stress. This leads to a distinctive sequence of color changes in the digits. Pallor indicates reduced blood flow due to oxygen deprivation, while erythema appears as reperfusion. RP can be primary, with no identifiable underlying cause, or secondary, associated with other conditions. These conditions include autoimmune diseases, most commonly systemic sclerosis, vascular diseases; and neurological conditions. While the exact cause of RP remains unclear, genetic and hormonal (estrogen) factors are likely contributors. The pathogenesis of RP involves a complex interaction between the vascular wall, nerves, hormones, and humoral factors, disrupting the balance between vasoconstriction and vasodilation. In primary RP, the vascular abnormalities are primarily functional. However, in secondary RP, both functional and structural components occur in blood vessels. This explains why digital tissue damage frequently occurs in secondary RP but not primary RP. Diagnosis of RP is primarily clinical. Recent advancements in imaging techniques have aided in diagnosis and monitoring, but nail fold capillaroscopy remains the gold standard for distinguishing between primary and secondary RP. If there are signs of acute ischemic injury, vascular imaging, particularly preoperatively, is crucial to rule out other vaso-occlusive conditions. Management of RP focuses on alleviating symptoms and preventing tissue damage. Vasodilator medications are the first-line treatment when general measures like warmth and stress management are not sufficient. Dihydropyridine calcium channel blockers (CCBs), such as nifedipine, are commonly used for vasodilation. Phosphodiesterase-5 inhibitors and prostaglandin analogs are alternative options for patients who do not respond to CCBs or have ischemic tissue damage. Bosentan, an endothelin-1 receptor antagonist, has shown effectiveness in treating and preventing digital ulcers, especially in patients with multiple ulcers. For severe cases, botulinum toxin injections or sympathectomy surgery can be used to control RP symptoms. However, botulinum toxin injections require repeated administration, and sympathectomy's long-term effectiveness is uncertain. Fat grafting is a promising surgical therapy for promoting healing and preventing tissue injury.
雷诺现象(RP)是一种以手指和脚趾间歇性过度血管收缩为特征的病症,由寒冷或压力引发。这会导致手指出现一系列独特的颜色变化。苍白表示因缺氧导致血流减少,而红斑则出现在再灌注时。RP 可以是原发性的,没有可识别的潜在病因,也可以是继发性的,与其他病症相关。这些病症包括自身免疫性疾病,最常见的是系统性硬化症、血管疾病和神经系统疾病。虽然 RP的确切病因尚不清楚,但遗传和激素(雌激素)因素可能是促成因素。RP 的发病机制涉及血管壁、神经、激素和体液因素之间的复杂相互作用,破坏了血管收缩和血管舒张之间的平衡。在原发性 RP 中,血管异常主要是功能性的。然而,在继发性 RP 中,血管中同时存在功能性和结构性成分。这就解释了为什么数字组织损伤在继发性 RP 中经常发生,而在原发性 RP 中则不会。RP 的诊断主要依靠临床。成像技术的最新进展有助于诊断和监测,但甲襞毛细血管镜检查仍然是区分原发性和继发性 RP 的金标准。如果有急性缺血性损伤的迹象,血管成像,特别是术前成像,对于排除其他血管闭塞性病症至关重要。RP 的治疗重点是缓解症状和预防组织损伤。当保暖和压力管理等一般措施不足时,血管扩张剂药物是一线治疗方法。二氢吡啶类钙通道阻滞剂(CCB),如硝苯地平,通常用于血管扩张。磷酸二酯酶-5 抑制剂和前列腺素类似物是对 CCB 无反应或有缺血性组织损伤的患者的替代选择。波生坦,一种内皮素-1 受体拮抗剂,已显示出在治疗和预防数字溃疡方面的有效性,特别是在患有多个溃疡的患者中。对于严重病例,肉毒杆菌毒素注射或交感神经切除术可用于控制 RP 症状。然而,肉毒杆菌毒素注射需要重复给药,交感神经切除术的长期有效性尚不确定。脂肪移植是一种有前景的手术治疗方法,用于促进愈合和预防组织损伤。