Raynaud's syndrome

Cold hands and feet are a common occurrence, usually blamed on the frigid weather, not dressing warmly enough or poor circulation. In most cases, a pair of mittens and some heavy socks will make you feel toasty again.

But at what point are cold hands a sign of more serious problems? Dr. Robert Spiera, director of vasculitis and scleroderma at the Hospital for Special Surgery in New York explains Raynaud's syndrome, a surprisingly common condition that underlies many cases of cold feet.

What is Raynaud's syndrome?
Raynaud's syndrome is a condition where people develop spasms in the blood vessels going to the hands or feet. These spasms are most often precipitated by exposure to cold, but they can also be caused by stressful situations.

It is something that is fairly common, especially in young women. Some estimates will say that as many as 10 percent of women have Raynaud's phenomena.

What are the symptoms of Raynaud's?
The hands or feet turn colors. The most typical thing would be first turning a whitish color, possibly followed by a phase where the hands can become very bluish and then a very reddish phase at the end. This is called the "French tricolor" changes of Raynaud's: white, blue and red.

These color changes are a result of the alterations in blood flow to the extremities. [The white color is caused by a lack of blood. The blue to red color is a result of a rush of blood to the hands.]

Should you be concerned about Raynaud's syndrome if you often get cold hands?
Raynaud's symptoms can fall within a spectrum in terms of the severity of the disease. There are people who are cold-sensitive, and their hands might turn a little white or feel a little bit cold in the cold weather; that's actually a normal physiologic response. But when people have Raynaud's, it's a more striking change, where they can actually see the color changes.

Why do people with Raynaud's get cold hands and feet?
Raynaud's is based on a normal physiologic response. If somebody is exposed to cold, the normal physiologic response is for your body to maintain core body temperature and prevent heat loss through the extremities. So, the body would clamp down on the peripheral vessels to have the blood go to the vital organs to maintain warmth.

But in people with Raynaud's, this normal response is exaggerated. For example, frostbite wouldn't be considered Raynaud's, but it's caused by the same response. In people who have Raynaud's, something like frostbite might happen much more readily or be more pronounced when it happens.

What causes Raynaud's phenomena?
There are families that are predisposed to having Raynaud's phenomena, but the symptoms of Raynaud's are usually indicative of some other disease. There are some autoimmune or connective tissue diseases that cause a higher rate and more severe form of Raynaud's.

For example, Raynaud's occurs in almost everybody with scleroderma, a chronic connective tissue disease. So, while some people just have Raynaud's syndrome alone with mild symptoms when they get very cold, in people with scleroderma the Raynaud's can be so severe that they develop injury to the tips of their fingers from the lack of blood flow. It becomes such a profound lack of blood supply that they actually can get ulcerations on the fingertips or even autoamputation (detachment) of the digits. In these patients with scleroderma and Raynaud's, the changes to the blood vessels can be seen; there's a thickening of the blood vessels which causes less room for the blood to flow.

How is Raynaud's diagnosed?
Raynaud's is really a clinical description. The only way to diagnose Raynaud's is by getting a good history from the patient and asking them to describe what happens to their hands. If the patient says, "Doctor, on cold exposure or during a stressful situation, my hands turn white and maybe little bluish," it's really just that description that would allow the doctor to diagnose Raynaud's.

Sometimes we'll just see it happen right in front of us. A patient gets nervous as a new doctor walks into the room, or they're in a cold exam room, and it will precipitate Raynaud's, so the doctor can observe it there.

How is the severity of the symptoms determined?
The severity would be determined by how much it bothers the patient, and how much it interferes with their daily life. That really depends on the individual patient and daily demands.

For example, I had a young woman who was a surgical resident and would go into the cold operating room and have the symptoms of Raynaud's there. It really wasn't necessarily a more severe form of Raynaud's than maybe somebody else's, but it interfered with her life more.

I would say it's significant if people are developing attacks where the white phase lasts more than 15 minutes. That really raises a flag that it might be serious Raynaud's, because during that white phase the fingers are not getting blood. That person is probably at higher risk for actually having injury to the tissues in their fingers or feet (but more often fingers) from the Raynaud's. This prolonged white phase signals more serious Raynaud's, and also would make me delve more deeply into whether this might be Raynaud's in the context of a more serious autoimmune or connective tissue disease like scleroderma.

What other diseases are associated with Raynaud's phenomena?
We seem to find that patients with autoimmune diseases have a higher frequency of Raynaud's than the general population. Scleroderma has the strongest association. In phospholipid syndrome, a person has certain blood proteins that predispose them to sluggish blood flow or blood clots that may show up as Raynaud's. We also see Raynaud's in patients with lupus.

Is this often the first sign of a more serious autoimmune problem?
Well, it can be. But more often than not, it's not going to be representing an autoimmune problem. So if somebody calls me up and says, "You know, I just met this young woman and she has a ten-year history of Raynaud's, and it's not changing, " I don't even think I need to see that person as a rheumatologist. But if someone's Raynaud's has suddenly gotten much, much worse, or somebody is 30 and is developing new Raynaud's, it can be a sign of an underlying connective tissue disorder brewing.

But I would say the most common consultation is to a young person with new Raynaud's. Do they have another disease or not? More often than not, they don't. But on the other hand, it is fair to look at that as something that should be at least evaluated, at least by their internist, if they develop new Raynaud's.

What does treatment consist of?
Once Raynaud's is diagnosed, how it is treated depends on how much it's interfering with the patient's life. There are some people with Raynaud's phenomena where they notice it, but it doesn't particularly bother them. So, beyond reassuring myself that it is nothing more serious, maybe that person doesn't need any treatment at all.

If a person is uncomfortable with it, there are a few very basic things to do. Maintaining core body temperature warmth is important. People with Raynaud's intuitively recognize, "Gee, I'd better wear gloves more often," but it's not just wearing gloves, it's also keeping a sweater on to keep your core body temperature up.

There are also medications we sometimes use in people who have very bad Raynaud's, or when it is interfering with their quality of life. One class of drugs used is called calcium channel blockers, like Procardia (nifedipine) or Norvasc (amlodipine), and are usually used as high blood pressure medicines. For Raynaud's they dilate the blood vessels and improve circulation. It's important to note that these drugs are not going to completely eliminate Raynaud's, but if they were having ten episodes a week, maybe they'll have four episodes a week. The drugs will decrease the frequency and often the severity of attacks.

Avoiding smoking is probably the most important thing for people with Raynaud's to do. You can actually give somebody a cigarette and precipitate a Raynaud's episode. Smoking causes spasm of the blood vessels.

Do you need to see a rheumatologist for an accurate diagnosis?
I think most rheumatologists would be comfortable with evaluating Raynaud's, but a lot of internists would be very capable of dealing with new Raynaud's as well. I think as rheumatologists, we deal with it a lot, and we're very familiar about the other things we should be thinking about in the context of new Raynaud's.

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