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Raynaud’s Phenomenon

What is Raynaud’s Phenomenon?

Raynaud’s phenomenon is a condition where the blood vessels in fingers and toes (and occasionally other areas) constrict upon exposure to cold, causing discoloration of the digits.

Disease vs. syndrome

There are 2 types of Raynaud’s phenomenon: primary Raynaud’s phenomenon or Raynaud’s disease, where symptoms are not associated with another disease, and secondary Raynaud’s phenomenon, or Raynaud’s syndrome, where symptoms are connected to another disease. Primary Raynaud’s phenomenon is not uncommon in young women.

  • Raynaud’s disease:
    • idiopathic. The cause is unknown
    • more common in young women
    • usually milder
  • Raynaud’s syndrome:
    • secondary to another disease
      • connective tissue diseases- systemic lupus erythematosus (SLE), scleroderma, rheumatoid arthritis, Sjogren’s syndrome, mixed connective tissue disease, etc.
      • Medications- beta-blockers, Bromocriptine, sulfasalazine, cyclosporine, some chemotherapies, others
      • Cryoglobulinemia
    • often more severe and may be more difficult to treat

 

What happens in Raynaud’s phenomenon:

The small blood vessels in the skin normally play a role in controlling the body’s temperature. They do this by constricting when it’s cold and reducing blood flow to the skin, therefore minimizing the loss of heat through the skin. They also dilate when it’s hot which increases the blood flow to the skin and helps lose some of the body heat. The blood vessels’ constriction and dilation is regulated by the sympathetic nervous system. Changes in the temperatures causes the sympathetic nervous system to fire signals to the blood vessels and direct them to either constrict or dilate. Interestingly, emotional stress can also cause a similar triggering of the sympathetic nervous system; which explains why people may have cold or sweaty hands when they are stressed.

In people with Raynaud’s phenomenon, the vessels have an abnormally exaggerated response to the sympathetic nervous system’s signals from cold weather.

Symptoms:

Although Raynaud’s phenomenon most commonly affects the blood vessels supplying the fingers, it can also involve the feet as well as the ears, nose, face, and rarely, other areas. The classic Raynaud’s phenomenon attack resembles the colors of the United States flag: white, red, and blue. Initially, when the blood vessels constrict, the fingers may turn white from the lack of blood flow. The attack may involve one or more fingers and may happen in one or both hands
simultaneously. The most commonly involved fingers are index, middle and ring fingers. The white discoloration sometimes does not involve the entire finger, and one may see a distinct border between the areas of white and pink in the same finger. If the constriction is severe or prolonged and the oxygen supply in the blood is running low, the digit may turn blue/purple. When the fingers are warmed up and the vessels dilate again, the fingers may become red, indicating a rush of blood flow to the skin.

  • white: decreased blood flowRaynaud
  • blue: lack of oxygen
  • red: blood flow re-established

 

 

During an attack of Raynaud’s phenomenon, the fingers or toes usually feel cold and they may be painful or numb. If the attacks are severe and prolonged, lack of blood supply to the area can eventually lead to death of the tissue (ulcers, necrosis, and gangrene). In severe cases, usually in people with Raynaud’s syndrome, tissue injury may be irreversible leading to loss of the finger or toe.

 

Diagnosing Raynaud’s phenomenon

Raynaud’s phenomenon is diagnosed clinically, based on one’s description of the fingers’ color change upon exposure to cold. When I evaluate someone for Raynaud’s phenomenon, my primary goal is to determine if the disease is primary or secondary. Primary Raynaud’s tends to be milder and responds better to conservative treatments. Secondary Raynaud’s (or Raynaud’s syndrome) can be more severe and more difficult to control. In addition, it can have implications depending on the disease it is associated with. For example, Raynaud’s symptoms associated with scleroderma can be associated with pulmonary hypertension, a very serious lung disease.

Blood tests are done in evaluation of Raynaud’s to rule out possible secondary diseases (see above). A rheumatologist may also do a nailfold capillary test, which involves looking closely at the small blood vessels around the nails. Normal blood vessels are thin and straight, but in severe Raynaud’s then can get inflamed, dilated, and tortuous. Recent studies have shown a correlation between abnormal nailfold blood vessels and development of pulmonary hypertension in people with scleroderma.

 

Treatment of Raynaud’s phenomenon

In milder cases, simple lifestyle changes may be all that one needs to control the disease. The more severe cases are treated with medications to prevent necrosis of digits and gangrene.

 

Keep fingers and toes warm

This may mean wearing socks more often and carrying a pair of gloves in your purse and using gloves to reach into a cold freezer. Keeping the entire body warm will also help warm the extremities- jackets, hats, scarfs. Running warm (but not hot) water over the affected fingers.

Stop smoking

Nicotine causes the blood vessels to constrict therefore worsening Raynaud’s.

Beware of medication reactions

Some medications can cause blood vessels to constrict. Some of these medications include beta-blockers (that lower blood pressure), pseudoephedrine (decongestants), among others.

Medical treatment

These are usually used in more persistent cases of Raynaud’s phenomenon. These medications work by dilating the blood vessel, helping restore blood to the extremities.

  • Calcium channel blockers– ex. Amlodipine, Nifedipine, Diltiazem
  • Topical nitroglycerine– ex. Nitrobid, Nitropaste
  • Other blood pressure medications– ex. Prazosin, Losartan
  • Phosphadiesterase inhibitors– ex. Sildenafil (Viagra), Pentoxyfylline (Trental), Cilostazole
  • Prostaglandins– ex.Prostacyclin

Procedural/Surgical treatment

In severe cases that are refractory to medications, surgical procedures are used to minimize injury.

  • Sympathectomy– cutting the nerves of the sympathetic nervous system that supply the blood vessels of the fingertips can their constriction
  • Amputation– If the damage is not reversible and not responsive to treatment, surgical amputation may be necessary.

Alternative treatments

Some nutritional supplements and herbs may help control milder cases of Raynaud’s phenomenon.

  • Ginko biloba
  • Fish oil
  • Vitamin D
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Living with rheumatoid arthritis- the 7 golden rules

Now that we have effective medications to treat this disease, living with rheumatoid arthritis is not as scary as once it may have been. You may still want to make a couple of adjustments in your lifestyle to help control the symptoms and prevent worsening of the disease. These are the 7 golden rules to help you live easier with rheumatoid arthritis:

 

1) Rest as needed

Inflammation associated with rheumatoid arthritis can cause significant fatigue. If your disease is active, you may need intermittent periods of rest during your day.

 

2) Exercise!

Staying active is an essential components of living with rhuematoid arthritis. With prolonged inactivity, joints will stiffen and muscles will become weak. This can actually exacerbate the pain in rheumatoid arthritis. Therefore it is a good idea to exercise your joints and muscles. If your arthritis is active, you may engage in milder forms of exercise until your disease is under better control. Many people with arthritis find it easier to exercise in the pool- swimming, aqua-aerobics, etc. Walking and biking are other, generally well tolerated forms of exercise. If you find that a particular exercise causes pain and swelling in your joints, opt for a different type of exercise instead.

3) Eat a healthy diet

Although there is no specific diet for rheumatoid arthritis, eating healthy, well balanced meals can be beneficial. A diet low in saturated fat and high in greens will help keep your heart healthy. While a healthy diet is important for everyone, it is especially crucial in people with rheumatoid arthritis, who are at higher risk for developing heart disease. If you are overweight, losing weight will lessen the load on your joints and help ease some of the pain.

 

4) Protect your joints

You may find that wrapping a joint with splints or braces can provide more stability to the joint and ease your pain. If you have arthritis
in your hip or knee, using a cane or a walker can help unload some of the weight. Nowadays, many assistive devices make living with rheumatoid arthritis easier. Some examples include:

  • Electrical appliances (can openers, power tools)Arthritis gadgets
  • Use of velcro instead of zippers or buttons
  • Padded handles for pots/pans, toothbrush, pen, keys, for easy grip
  • Elevated seats or chair legs
  • Larger door/cabinet/drawer handles
  • Many more

 

These devices may be available in pharmacies, surgical supply stores, or online. Here are just a few examples of places you can look:
http://www.aidsforarthritis.com/catalog/index.html
http://www.arthritissupplies.com/around-the-house.html

4) Consider Supplements

While herbal supplements and vitamins do not play a major role in rheumatoid arthritis, data suggests some benefits for several supplements:
People with rheumatoid arthritis are at risk for developing osteoporosis; therefore vitamin D supplements may help prevent this. Fish oil is rich in omega-3 and may have anti-inflammatory properties Borage seed oil and Turmeric also have mild anti-inflammatory effects.

 

5) Keep your followup appointments

Rheumatoid arthritis is a chronic disease. You will most likely need regular visits with your rheumatologist or primary care physician to adjust your medications, monitor your disease, and prevent complications from rheumatoid arthritis.

 

6) Get your vaccines in order

Like in many chronic conditions, people with rheumatoid arthritis are encouraged to have yearly flu shots. Pneumonia vaccine is also recommended. If you are on medications that can lower your immune system, you should also get vaccinated for shingles.

 

7) Stop smoking!

We now have sufficient data that shows smoking is not only a risk for developing rheumatoid arthritis, it also can exacerbate the disease and interfere with the medications that are used to treat it.

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Rheumatoid Arthritis Quiz

Test your knowledge of rheumatoid arthritis with this quiz!

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Do Rheumatoid Arthritis treatments increase cancer risk?

Whether or not biologic drugs increase the risk of cancer in people with rheumatoid arthritis has been the subject of much research and concern in the past few years.

Biologic drugs work by targeting and disabling a specific component of the immune system. Since rheumatoid arthritis is an autoimmune disease, targeting the immune system has been a very effective way of treating the disease. But the question has been, what exactly is the role of the immune system in controlling and fighting cancer? And what happens if we interfere with this function?

We do know that patients with rheumatoid arthritis in general are at higher risk of getting certain malignancies than the general population. Specifically, they are at higher risk of developing lymphoma, leukemia, lung cancer, and skin cancer. Non-Hodgkin lymphoma is the most common malignancy in rheumatoid arthritis patients. This risk is directly related to the degree and duration of inflammation. In other words, the worse the disease, the higher risk of malignancy.

We first started using biologic drugs for treatment of rheumatoid arthritis in 1996. A class of biologic drugs named TNF-inhibitors was the first to be used, and it is still first line biologic treatment for treatment of rheumatoid arthritis. The results have been dramatic, with miraculous improvements in pain, swelling, and prevention of joint deformity.  But in 2003 the FDA noted 6 cases of lymphoma in over 6000 patients that were treated with TNF-alpha inhibitors. The TNF inhibitors available at that time were Enbrel, Humira, and Remicade. So the concern was born:

 

Do biologic drugs increase risk of cancer in patients with rheumatoid arthritis?

 
In trying to answer this question, we have learned a few things. First, that almost all the cancers reported in 2003 seemed to have happened in the first year of the TNF inhibitor use. In fact, if one looks at cancer risk over several years, the overall incidence seems to be the same in people who used biologics and those who did not. This can suggest that the drug did not actually cause the cancer, but unmasked the growth of one that was already there, too small to be detected at the time of starting the drug.

Second, if cancer risk is greater in patients with worse rheumatoid arthritis, and these patients were the ones who needed the stronger TNF inhibitor drugs to treat their disease, it is not surprising that they developed the malignancy while those patients with milder disease who did not need the stronger drugs did not.

Finally, the risk of cancer is still very low- both in general population and in patient with RA. This has made investigating the difference between the two groups even more difficult, since most studies of these drugs do not have enough patients developing cancer to power the statistics. So, we have relied on observational studies and metanalyses to answer this question. In fact, there have been multiple such studies in the past few years. And the answer? These studies showed that there is a higher rate of malignancy in patients with rheumatoid arthritis than in the general population; however the risk is the same in patients receiving biologic therapies as those who are not.

 

The 2012 American College of Rheumatology conclusions on this subject are:

  1. RA patients are at increased risk of lymphoma and lung and skin cancer
  2. Biologic-treated RA patients have similar rates cancer as do non-biologic treated RA patients
  3. RA patients on biologics with a prior history of most cancers are not risk of cancer recurrence

 

Reference:

http://www.rheumatology.org/

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