The prevalence of Raynaud’s phenomena varies widely between populations. In the US, it is estimated that up to 200,000 people are affected. A higher prevalence among other populations, such as France, is found (3). Raynaud’s phenomenon can be either a primary phenomenon or secondary to various underlying medical disorders and drug-related conditions.

Vasospastic angina, previously called Prinzmetal or variant angina, is a form of abnormal vascular response to various stimuli. Prinzmetal et al (4) initially described a clinical syndrome that manifested as rest angina associated with ST-segment elevation that promptly responded to sublingual nitrates. Since this differs from classical angina described by Heberden (effort angina associated with ST depression) (5), he referred to it as variant angina.

The correlation between vasospastic angina and Raynaud’s has been explained previously in literature (6,7). Miller et al (6) reported a high prevalence of Raynaud’s phenomenon in patients with vasospastic angina occurring at a rate of 24% in patients with variant angina as compared to 3% and 5% in controls (p-value<0.01).  Hence vasospastic angina in some patients is a manifestation of a generalized vasospastic disorder and could be associated with migraine, Raynaud’s phenomenon, and aspirin-induced asthma (8).

Coronary vasospasm can occur in patients with normal coronary arteries as well as in patients with atherosclerosis. However, it is still poorly understood whether coronary vasospasm can promote atherosclerotic alterations or whether the latter may predispose to spasm (9).

The patient described in our case has a typical history of Raynaud’s phenomenon diagnosed clinically, along with recurrent attacks of chest pain relieved by nitrates that were thought to be due to vasospastic angina. However, coronary angiography revealed atherosclerotic narrowing with 80% occlusion of the right coronary artery. This finding was similar to an autopsy result in one of the patients described in Prinzmetal et al paper (4), which revealed that the right coronary artery (which presumably had caused resting angina with ST elevation in inferior leads) had an 80% stenosis. This observation led to the conclusion that coronary spasm was associated with atherosclerosis. Later it was found that coronary spasms can also occur in patients with angiographically normal coronary arteries (10).

Recent experimental studies demonstrate that several endothelium-derived factors mediate vascular smooth muscle cell tone and that damaged endothelium may play a major role in the pathogenesis of vasospasm (11). Endothelial damage may occur even in the early stages of atherosclerosis. Zeiher et al (12) indicated that abnormal vasoconstriction was observed in early atherosclerotic sites in patients without variant angina using intra-coronary ultrasound. Yamagishi et al (13) reported that early stages of atherosclerosis were observed by intracoronary ultrasound at the site of spasm in patients with variant angina, even in the absence of an angiographically significant coronary disease. However, these results were challenged by a porcine model of spasm, indicating that endothelial vasodilator function was preserved at the spastic site (¹⁴); it remains to be elucidated.

It was suggested that endothelial dysfunction with decreased nitric oxide release and reduced bioavailability combined with vascular smooth muscle cell hyperreactivity might be important in developing coronary spasm. However, endothelial dysfunction alone might not be sufficient to explain vasospastic angina (15). Therefore, more studies need to be done to understand the pathophysiology associated with the vasospasm associated with Raynaud’s disease.