Raynaud's Disease
October is Raynaud’s Awareness Month, and for many people with Raynaud’s (pronounced Ray-NODES), the chilling, uncomfortable truth is - living with it can be incredibly chilling and uncomfortable.
Five to ten percent of the population live with this blood flow condition that affects the body’s ability to send blood to the extremities. Primarily affecting the hands and feet, it causes the area to become very cold and can change the color of the hands or feet to white, gray, blue, or red. It affects around 25%-60% of SLE patients and is considered the most common SLE cardiovascular issue. The lack of circulation can cause numbness or tingling, and in severe cases, cause ulcers and sores, and (at its worst) lead to gangrene and partial amputation.
Raynaud’s Disease can be a “primary” disease with no associated disorder. It can also occur as a “secondary” condition (often referred to as Raynaud’s Phenomenon) related to other diseases, such as scleroderma, rheumatoid arthritis, and lupus.
This blog is intended to shed light on a disease that, over a hundred years after its discovery, is still very mysterious and has no exact known cause or cure.
History of Raynaud’s
Raynaud’s was named after the French physician Maurice Raynaud, who described the condition in 1862 in his doctoral thesis. He characterized a set of symptoms that caused “Intermittent pallor and cyanosis of the extremities” and “Cold-induced vasospastic attacks of the fingers,” which could lead to gangrene in severe cases.
In 1929, Sir Thomas Lewis set out to challenge the enigma behind Maurice Raynaud’s theory - stating an over-reactivity of the sympathetic nervous system caused it. Lewis believed it was not nervous system related, but rather, from a “local fault” of the digital artery. Both opinions were considered throughout the early twentieth century until the discovery of cold-induced cutaneous arterial vasoconstriction (narrowing of the blood vessels), bridging the gap between both theories.
1932, the first set of classification criteria to differentiate primary Raynaud’s disease from secondary Raynaud’s phenomenon was published. Since then, rheumatologists, vascular specialists, and internists have been treating patients worldwide with this condition in its primary and secondary forms.
Symptoms
Raynaud’s symptoms can vary from mild to severe. Most individuals experience the following:
Cold fingers and toes; the nose and ears are also often colder than normal.
Color changes in the skin (white, gray, blue) in response to cold or stress.
Numbness or tingling in the fingers and toes (can be on the ears, nose, and nipples as well).
Redness, stinging or throbbing pain upon warming or stress relief.
In severe cases (most commonly in lupus patients who also have scleroderma, called systemic lupus with scleroderma overlap syndrome): sores or ulcers in the tips of fingers and/or toes.
More severe cases can have a decrease in the spongy soft tissue at the ends of the fingers (the finger pulp) or a tapered appearance of the tips of the fingers.
Changes in the fingernails or toenails.
What is a “Raynaud’s Attack?” During an attack of Raynaud's, affected areas of your skin usually first turn white or gray. Then, they often turn blue and feel very cold and numb. As you warm up and your circulation improves, the affected areas may turn red, throb, tingle or swell. It can take approximately fifteen minutes for the blood vessels to release and return proper blood flow to the affected area.
The classic color triad of a Raynaud’s attack is pallor (white or grey) then bluish/purple then red. However, many people who have Raynaud’s have only one or both of the pallor or bluish phases. Red fingers alone are not consistent with Raynaud’s.
Raynaud's attacks can happen at any time or any place; and commonly are brought on by normal everyday events, like being in the grocery store frozen section, holding a cold beverage at a restaurant, or even showering.
It can be hard to avoid all things that might make your hands cold. But try your best to carry mittens (wool is best), keep your hands in your pockets, and invest in some hand warmers.
If you feel an attack coming, take this advice from Dr. Thomas:
"To stop a Raynaud's attack, wiggle your fingers, rub your hands together, run them under warm (not hot) water, put them under your armpits, or whirl your arms around like windmill to increase blood flow."
- Dr. Don Thomas, The Lupus Encyclopedia, p. 236
Lupus and Raynaud’s
If you are living with lupus or another disease, it can be scary and frustrating to be told you may also have Raynaud’s. Raynaud’s is a common problem in lupus and other autoimmune conditions like scleroderma, rheumatoid arthritis, and Sjögren’s Disease.
As mentioned above, your rheumatologist will oversee your care if you have lupus and develop Raynaud’s. Your doctor may suggest you implement these tips to help prevent the frequency and severity of the symptoms.
Keep the extremities warm, wear mittens or gloves when it is cold outside or when handling cold items.
Stop smoking.
Wear comfortable shoes and wool socks.
Avoid caffeine.
Avoid medications that cause tightening of the blood vessels (like decongestants and amphetamine-like diet pills).
Treatment
Allowing mild attacks to happen that don't interfere with daily functions and are not painful may be doable. However, if attacks are painful and moderate to severe, it is important to talk to your doctor about possible treatments.
Treatments for Raynaud’s may include:
Calcium channel blockers: These medications help dilate (enlarge) the blood vessels (also called vasodilators) thereby increasing circulation to the extremities. Examples: nifedipine and diltiazem. Most experts consider these the most important drugs to try before considering those below.
Alpha Blockers: These medications block the effects of the hormone norepinephrine. which usually causes blood vessels to constrict (tighten up). They are also a type of vasodilator. Examples: of this type of medication are Minipress (prazosin) and Cardura (doxazosin).
Other vasodilators: These medications relax the walls of the blood vessels. Examples: Viagra (sildenafil), Cozaar (losartan), Prozac (fluoxetine), and nitroglycerin.
Surgical intervention: In very rare cases where the blood flow has been completely blocked, gangrene may be a serious issue. Partial amputation to remove the affected and damaged tissue may be the only option.
Alternative therapies: Acupuncture, biofeedback, and fish oil supplements may help slow down the narrowing of the blood vessels. Low-dose aspirin can thin out the blood, allowing easier blood flow in the tiny fingertip blood vessels.
Pain relief: Pain relief is an important part of treatment. It is important to tell your doctor ASAP if you have new or worsening pain from your Raynaud's because the pain can make it worse. Tylenol (if there is no lives disease or alcoholism), nonsteroid anti-inflammatory drugs (if there is no contraindications), or tramadol may help.
If you are considering trying an alternative treatment option, you must speak with your doctor first. Only your physician will know of any potential drug interactions or side effects of these alternative therapies.
Outlook
While Raynaud’s is rarely life-threatening, complications can develop without addressing the symptoms. Working with your physician to find the right treatment plan specific to your needs is the best action. Sometimes, Raynaud’s improves, or symptoms simply disappear. Every person with Raynaud’s is unique.
For more updated information on Raynaud’s please visit : https://www.raynauds.org/
Article By:
Kelli Roseta
Reviewed and amended by: Donald Thomas, M.D., FACP. FACR, RhMSUS
**All resources provided by this blog are for informational purposes only, not to replace the advice of a medical professional. Kelli encourages you to always contact your medical provider with any specific questions or concerns regarding your illness. All intellectual property and content on this site and in this blog is owned by morethanlupus.com. This includes materials protected by copyright, trademark, or patent laws. Copyright, More Than Lupus 2023.
Sources:
https://link.springer.com/chapter/10.1007/978-1-4939-1526-2_1
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5700788/
The Lupus Encyclopedia, By Dr. Don Thomas, Johns Hopkins Press, p. 234-237