Raynaud's Disease
What Is Raynaud's Phenomenon?
Raynaud’s phenomenon is a condition that affects the blood vessels in the extremities—generally, the fingers and toes. It is characterized by episodic attacks, called vasospastic attacks, in which the blood vessels in the digits (fingers and toes) constrict (narrow), usually in response to cold temperatures and/or emotional stress. When this condition occurs on its own, it is called primary Raynaud’s phenomenon. When it occurs with another condition such as scleroderma or lupus, it is called secondary Raynaud’s phenomenon.
Who Gets Raynaud’s Phenomenon?
Although people of any age can have Raynaud's phenomenon, the primary form typically begins between the ages of 15 and 25. Women are more likely than men to have Raynaud's phenomenon. It appears to be more common in people who live in colder climates. This is likely true because people with the disorder have more Raynaud’s attacks during periods of colder weather. Although estimates vary, most studies show that Raynaud’s phenomenon affects about 3 percent of the general population. For most, the symptoms are mild and not associated with any blood vessel or tissue damage. Most people with Raynaud’s phenomenon have the primary form, which is not associated with any underlying disease. In fact, in these individuals it is thought to be an exaggeration of normal responses to cold temperature and/or stress.
When Raynaud’s phenomenon is caused by or associated with an underlying disease, it is referred to as secondary Raynaud’s phenomenon. Secondary Raynaud’s phenomenon tends to begin later in life than the primary form, typically after 35 to 40 years of age. It is common for patients with a connective tissue disease to have Raynaud’s phenomenon. It occurs in more than 90 percent of patients with scleroderma, and in about 30 percent of patients with systemic lupus erythematosus and with Sjögren’s syndrome. Secondary Raynaud's phenomenon may also be associated with exposure to vibrating tools such as jackhammers, which cause trauma to the hands and wrists. And it may be linked to certain drugs, such as chemotherapy agents, or to chemicals such as vinyl chloride.
What Is the Difference Between Primary and Secondary Raynaud's phenomenon?
In medical literature, primary Raynaud's phenomenon may also be called idiopathic Raynaud’s phenomenon, primary Raynaud’s syndrome, or Raynaud’s disease. There is no known cause for primary Raynaud’s phenomenon. It is more common than the secondary form and often is so mild the patient never seeks medical attention. It generally is an annoyance that causes little disability. Secondary Raynaud’s phenomenon is a more complex and serious disorder. The most common cause of secondary Raynaud’s phenomenon is connective tissue disease. The condition most commonly occurs with scleroderma or lupus, but is also associated with Sjögren’s syndrome, dermatomyositis, and polymyositis. Some of these diseases reduce blood flow to the fingers and toes by causing blood vessel walls to thicken and the vessels to constrict too easily.
Other possible causes of secondary Raynaud’s phenomenon are carpal tunnel syndrome and obstructive arterial disease (blood vessel disease). Some drugs are also linked to Raynaud’s phenomenon. They include beta-blockers, such as Lopressor1 or Cartrol, used to treat high blood pressure; ergotamine preparations, such as Cafergot or Wigrane, used for migraine headaches; certain agents used in cancer chemotherapy; and drugs, such as over-the-counter cold medication and narcotics, that cause vasoconstriction. People in certain occupations may be more vulnerable to secondary Raynaud’s phenomenon. Some workers in the plastics industry who are exposed to vinyl chloride, for example, develop a scleroderma-like illness, of which Raynaud’s phenomenon can be a part. Workers who operate vibrating tools can develop a type of Raynaud’s phenomenon called vibration-induced white finger. Severe cases of Raynaud’s phenomenon—usually of the secondary form—can lead to problems such as skin ulcers (sores) or gangrene (tissue death) in the fingers and toes, which can be painful and difficult to treat.
What Is the Treatment for Raynaud's phenomenon?
The aims of treatment are to reduce the number and severity of attacks and to prevent tissue damage and loss of tissue in the fingers and toes. Most doctors are conservative in treating patients with primary Raynaud's phenomenon because they do not get tissue damage. For these patients, doctors tend to recommend nondrug treatments before moving onto medications. For patients with secondary Raynaud's phenomenon, medications are more often prescribed, because severe attacks with ulcers or tissue damage are more likely. In the most severe cases, Raynaud’s causes ulcers and serious tissue damage that does not respond to medications. Doctors may use a surgical procedure called a digital sympathectomy with adventitial stripping (which involves removing the tissue and nerves around the blood vessels supplying the affected digits). While this procedure may result in reducing symptoms and healing tissue, it only helps temporarily and therefore is reserved for difficult cases.
Credit: National Institute of Health.