Grader-Beck Thomas, Wigley Fredrick M
Division of Rheumatology, Johns Hopkins School of Medicine, 5200 Eastern Avenue, Suite 4100, Baltimore, MD 21224, USA.
Rheum Dis Clin North Am. 2005 Aug;31(3):465-81, vi. doi: 10.1016/j.rdc.2005.04.006.
Raynaud's phenomenon affects most patients who have mixed connective tissue disease (MCTD) and frequently represents the initial manifestation of the disease. It is the cutaneous symptom of a systemic vasculopathy that is characterized by intimal fibrosis and blood vessel obliteration that frequently leads to visceral involvement, particularly pulmonary hypertension. An association between Raynaud's phenomenon and the characteristic autoantibody in MCTD, anti-U1-RNP (ribonucleoprotein), is found across the spectrum of rheumatic diseases, including undifferentiated connective tissue disease, scleroderma, and systemic lupus erythematosus. Capillary nailfold examination represents a valuable tool to identify patients who are at risk for MCTD. The goal in the therapy of Raynaud's phenomenon in MCTD is to decrease the frequency of attacks, to prevent digital ulceration, and to limit progressive vascular damage. Therapeutic regimens include the traditional use of calcium channel blockers and novel vascular therapies.
雷诺现象影响大多数混合性结缔组织病（MCTD）患者，且常为该病的首发表现。它是一种系统性血管病的皮肤症状，其特征为内膜纤维化和血管闭塞，常导致内脏受累，尤其是肺动脉高压。在包括未分化结缔组织病、硬皮病和系统性红斑狼疮在内的各种风湿性疾病中，均发现雷诺现象与MCTD中的特征性自身抗体抗U1 - RNP（核糖核蛋白）之间存在关联。甲襞毛细血管检查是识别有MCTD风险患者的一项有价值的工具。MCTD中雷诺现象的治疗目标是减少发作频率、预防指端溃疡并限制进行性血管损伤。治疗方案包括传统使用的钙通道阻滞剂和新型血管疗法。
