Everyone knows the feeling of uncomfortably cold hands when the temperature drops, but some people feel it more than others. However, Raynuad’s disease is a whole other level.
People with Raynaud’s disease don’t just feel cold — the blood flow to their fingers is cut off so dramatically that their hands turn white, then blue, and can temporarily lose function.
“Raynaud’s is an exaggeration of a normal response to temperature,” explains Fredrick M. Wigley, MD, a Raynaud’s expert, rheumatologist, professor of medicine, and director of the Johns Hopkins Scleroderma Center in Baltimore. Dr. Wigley adds that the condition — also called Raynaud’s phenomenon or Raynaud’s syndrome — sometimes affects the toes as well, and occasionally the ears, nose, knees, and nipples.
In Raynaud’s the small blood vessels that supply the extremities with blood are overly sensitive. Exposure to cold — as well as feeling anxious or stressed — can make these vessels suddenly constrict to severely restrict blood flow, resulting in the discomfort and color changes characteristic of Raynaud’s.
“Grabbing a glass with ice in it, or your car’s steering wheel when it’s cold out can trigger an immediate attack,” says Elena Schiopu, MD, an associate professor of medicine at the University of Michigan and rheumatologist at the Michigan Medicine Rheumatology Clinic in Ann Arbor. “But people also get attacks if they get a chill elsewhere on the body, or if they feel stressed out.”
The Two Types of Raynaud’s
There are two types of Raynaud’s: primary and secondary. “Primary Reynaud’s tends to first occur around the onset of the teenage years,” explains Dr. Wigley, who adds that the condition affects more females than males. While the exact cause is unknown, in many cases another family member also has the condition, which suggests a genetic link that has yet to be discovered.
People with primary Raynaud’s are usually otherwise healthy, and medical exams to look for abnormal vascular changes don’t uncover any underlying condition. “The blood vessels are just a little temperamental, and clamp down for no good reason,” says Dr. Schiopu.
Primary Raynaud’s is considered more of an annoyance than a serious medical condition. “And it tends to get better as you get older,” says Dr. Wigley. Symptoms can be aggravated by smoking, repetitive force to the hands (for instance, using vibrating power tools), and certain medications. “For instance, in children who are given certain medications for attention deficit disorder, about 30 percent of them develop Raynaud’s,” he says. According to the American College of Rheumatology, approximately 10 percent of the general population has primary Raynaud’s.
Things are a bit more complicated with secondary Raynaud’s, which first emerges in adulthood, and is linked to an underlying disorder. “If you have the onset of symptoms after the age of 25 or 30, then there’s reason to think there might be a secondary cause,” explains Dr. Wigley. Often that cause is a rheumatic or autoimmune disease that disturbs the blood vessels.
The symptoms of secondary Raynaud’s can be more severe, and the condition more serious than in the primary form of the condition. “In secondary Raynaud’s the impact on function is huge, especially in colder climates,” says Dr. Schiopu. “People may feel tremendous pain that makes them unable to work, sleep, or concentrate. They can get digital ulcers from complete lack of blood supply to the fingers.” In rare cases, the ulcers can become infected or gangrenous, leaning to amputation.
How Raynaud’s Is Diagnosed
Rheumatologists are the doctors best equipped to diagnose Raynaud’s. When a patient comes in with symptoms, an evaluation will include a complete medical history, physical exam, and blood tests to determine if the Raynaud’s is primary or secondary. According to the American College of Rheumatology, one exam involves looking at the blood vessels below the fingernails (called nailfold capillaroscopy). These blood vessels are normal in people with primary Raynaud’s, but abnormal in people with secondary Raynaud’s.
Blood tests to detect other conditions can also help distinguish between primary and secondary Raynaud’s. In addition, a doctor may set up a cold challenge to see if the symptoms can be induced. Sometimes, just the anxiety associated with visiting the doctor can cause Raynaud’s symptoms during an office visit.
The Link Between Raynaud’s and Rheumatic Disease
Many rheumatic and autoimmune diseases that have been associated with secondary Raynaud’s. Some of the most common include:
Scleroderma: The connection between scleroderma and Raynaud’s is so strong that nearly 100 percent of people with scleroderma also experience Raynaud’s, according to Dr. Wigley. Raynaud’s symptoms are often what first bring people with scleroderma to the doctor, he adds.
Systemic lupus: “About a third of people with this condition also have secondary Raynaud’s,” says Dr. Wigley.
Sjogren’s syndrome: “About 30 percent of these patients also have Raynaud’s,” says Dr. Wigley.
Myositis (muscle inflammation) including dermatomyositis and polymyositis: “Also about 30 percent,” says Dr. Wigley.
Rheumatoid arthritis: The connection between this type of arthritis and Raynaud’s is still debatable. “Some surveys have shown that Raynaud’s is more common in people with RA, some not.” But Dr. Wigley adds that Raynaud’s in this population tends to be a little less severe.
Other conditions that have been associated with secondary Raynaud’s include some thyroid disorders and carpal tunnel syndrome.
Raynaud’s Treatment Options
For people with secondary Raynaud’s, treating the underlying condition — such as lupus or scleroderma — doesn’t necessarily make the Raynaud’s disappear. And there is no FDA-approved medication to treat Raynaud’s, although doctors do sometimes prescribe drugs such as calcium channel blockers (usually used to treat high blood pressure) that can improve blood flow to the extremities.
Other vasodilators, prostaglandins, and even antidepressants have been tried, and clinical trials are underway to devise more effective drug treatments. “And there’s been some success with biofeedback and cognitive behavioral therapy, too,” says Dr. Schiopu. “But those treatments are expensive and a bit complicated, and many people don’t have time for them.”
Many patients with Raynaud’s don’t need any special medication to prevent or treat the symptoms. “Not every patient with Raynaud’s needs drugs, but every patient with Raynaud’s needs non-drug treatment,” says Dr. Wigley. For most people, the key to controlling Raynaud’s symptoms is to recognize what triggers them — and make lifestyle changes to minimize the frequency and severity.
Lifestyle Changes to Control Raynaud’s Symptoms
Dressing to keep the body warm (and the temperature consistent) is key. “The body senses it’s cold even if the hands are warm,” says Dr. Wigley. Dressing to prevent Raynaud’s symptoms is more than simply wearing warm gloves; it’s just as important to wear layered clothing that keep the core warm, as well as a hat when necessary.
Dr. Schiopu, who practices medicine in chilly Michigan, advises patients to consider heated gloves and steering wheel warmers for their cars.
“Some of my patients go to Florida in the winter, says Dr. Schiopu. “Their Raynaud’s can actually get worse there because they’re frequently going from a very warm outside temperature to an air-conditioned indoors.”
Raynaud’s symptoms can occur anywhere in the country, at any time, such as when you enter a supermarket and shop in the frozen food aisle. Dr. Schiopu adds that over time her patients get very good at recognizing their Raynaud’s triggers and learning to work around them.
Quitting smoking, avoiding vibrating power tools, having a doctor evaluate the medications you take (to switch any that might aggravate Raynaud’s), and learning to recognize stress and anxiety triggers, are also helpful ways to prevent Raynaud’s attacks.
Surgery for Raynaud’s
In very severe cases, when lifestyle changes and medications aren’t enough to control the symptoms and patients are at risk of losing their fingers, a variety of surgical techniques are available. According to the Raynaud’s Disease Clinic at Harvard’s Brigham and Women’s Hospital, “One such innovative technique is to remove the sheath of nerves surrounding the hand’s blood vessels, thereby eliminating the nerves responsible for triggering vasospasms.”
Fortunately, for most people with Raynaud’s, lifestyle changes — and sometimes medication — will keep the condition well under control.