ABSTRACT

Small artery disease Approximately 90-95% of patients with upper extremity ischemia have small artery disease where the most common symptom is Raynaud’s syndrome.1,2,6 Raynaud’s syndrome is caused by vasospasm of the digital arteries and arterioles and is characterized by episodic digital ischemia provoked by stimuli such as cold or emotional stress (Figure 42.1). The affected digits undergo tri-color changes in which they initially turn white, followed by blue and then red, usually resolving in 10-60 minutes after removal of the inciting stimulus. The digits are usually normal between attacks. Raynaud’s syndrome has a female-to-male ratio of 4:1 and its typical onset is during the second and third decades of life. It is estimated that 5-15% of the healthy population is affected with this disorder. The vast majority of patients (89%) with Raynaud’s syndrome have primary Raynaud’s syndrome, which is also known as Raynaud’s disease. Raynaud’s disease should not be confused with secondary Raynaud’s syndrome which is also known as Raynaud’s phenomenon.2,6 Patients with primary Raynaud’s syndrome do not have evidence of arterial occlusive disease or signs of other systemic disorders. Primary Raynaud’s syndrome is not life threatening and patients usually lead normal lives. To confirm the diagnosis of primary Raynaud’s syndrome or Raynaud’s disease, patients must have Raynaud’s syndrome for more than two years without evidence of systemic disease.1,2,6

In contrast, secondary Raynaud’s syndrome is due to an underlying condition (Table 42.2). Patients with secondary Raynaud’s syndrome due to collagen vascular diseases are more likely to have arterial obstruction resulting in digital ulceration and gangrene.1,6 In addition, Raynaud’s syndrome often precedes the underlying systemic disease by 10-20 years, therefore once a diagnosis of Raynaud’s syndrome is made, secondary Raynaud’s syndrome must be confirmed or excluded.6 The major differences between primary and secondary Raynaud’s syndrome are summarized in Table 42.3.