Raynaud’s syndrome is a disorder where abnormal spasm or vasoconstriction occurs in the small digital arteries of the fingers and toes while the major peripheral pulses remain intact. Spasm of these small vessels prevents adequate arterial blood flow from reaching the tissue of the digits and causes them to turn pale. As the spasm continues, the blood retained in the digits gets further depleted of its oxygen content and the digits turn a blue hue. The lack of oxygen (ischemia) leads to a reflex response where the spasm relaxes and the flow is once again restored. This causes a red color to appear due to the temporary excess of flow within the digits. The magnitude and duration of the spasm effect can be variable and these cycles tend to occur repeatedly.
There are two basic types of Raynaud’s syndrome: primary and secondary. Primary Raynaud’s is where the cause cannot be attributed to any other type of medical condition and is considered unknown. Characteristically, there is no evidence of atherosclerotic vascular disease in these individuals. Over 90% of Raynaud’s patients have a primary etiology. The condition is primarily considered benign and in very rare circumstance will the spasm lead to tissue loss (ulceration or gangrene). The disease occurs in up to 15% of otherwise normal people and affects women four times more frequently than men. Symptoms can begin as early as the teenage years or up into one’s thirties. There is a higher incidence of symptomatic individuals in cooler climates. Symptoms typically affect both hands and/or feet and peripheral pulses remain intact. To actually make the diagnosis of primary Raynaud’s syndrome, patients should have vasospasm symptoms for at least 2 years and have no other problem that could explain their symptoms. Raynaud’s does not appear to have a genetic predisposition.
The diagnosis is typically suggested by the patient’s history of coolness, numbness or tingling, or pain in the hands or fingers with cold exposure. The thumb tends to be spared from involvement for reasons which are not known. Symptoms are brought on by cold exposure in over 95% of cases while emotional distress is thought to initiate symptoms less than 5% of the time. Non invasive vascular testing can be helpful by measuring the blood flow wave form of the digital arteries both at rest and after immersing the fingers in cold water. Patients with Raynaud’s symptoms are found to have normal blood flow wave forms at rest, but after cold water exposure, the digital wave forms will become dampened or entirely flat line.
Secondary Raynaud’s syndrome is where the vasospasm symptoms are due to an identifiable underlying disease process. The most common cause of secondary Raynaud’s syndrome is collagen vascular disease, in which over 90% of symptomatic patients are found to have scleroderma. Other causes of secondary Raynaud’s syndrome include Lupus, Sjogren syndrome, CREST syndrome, polymyositis, and rheumatoid arthritis. Less common causes are upper extremity atherosclerotic occlusive disease, including arterial emboli and Buerger’s disease (as the result of smoking); trauma from frostbite or the use of tools with lots of vibration (jack hammers); drugs including beta blockers and oral contraceptives; and other conditions such as thoracic outlet syndrome, hypothyroidism, and kidney disease.
Patients with collagen vascular disease and Raynaud’s syndrome have a more severe symptomatic picture including development of ulcers or gangrene of their digits. Blood tests such as sedimentation rate (ESR) and antinuclear antibodies (ANA) can assist in establishing a diagnosis of connective tissue disease. An elevated ESR is a general marker of a systemic (system wide) inflammatory process which is not specific for any one diagnosis while antinuclear antibodies are detected in conditions where the body’s immune system attacks its own cellular components. A homogeneous ANA pattern can be seen in lupus, a speckled pattern in scleroderma, and antibodies against specific components of the cell nucleus found in CREST syndrome. In patients with tissue loss or gangrene, performing an angiogram may be helpful in identifying other types of peripheral vascular disease and the extent of the vascular compromise.
The treatment of Raynaud’s syndrome is primarily directed at reducing symptoms. Keeping the central core of the body warm means more heat will be transferred to the periphery and reduce or eliminate hand and feet vasospasm. Patients are advised to dress in layers and wear gloves. Smoking, caffeinated beverages, and chocolate are known to cause vasoconstriction and their use should be minimized or avoided all together. In patients with severe pain or complications such as digital vessel thrombosis (clotting), or ulceration of the skin; medical treatment with vasodilatation agents should also be considered. Most of the medications are commonly used for the treatment of high blood pressure or heart disease. Commonly used medications include calcium channel blockers and sympathetic blocking agents. Antiplatelet agent (aspirin) are also recommended to reduce the chance of intravascular thrombosis during the low flow aspect of the vasospastic attack. Treatment of secondary Raynaud’s syndrome also includes addressing the primary underlying disease condition as well.