Raynaud’s phenomenon


The primary form of Raynaud has to do with the blood supply to the fingers, toes or nose. It is due to the small blood vessels in the fingers, toes or nose that “spasm”, thus decreasing the blood flow to the fingertips, toes or nose. The blood vessels act as a heat regulator, so if you have warm hands, you radiate a lot of heat through your hands and fingers and the blood in the blood vessels flows well. If you are feeling cold, your body will try to retain the heat by restricting the release of heat and narrowing the blood vessels. You will then get white ‘numb‘ or ‘dead‘ fingers, toes or nose that will turn blue-purple later on and become painful and red or purple in a warm environment. In addition to that ‘dead‘ feeling, tingling can also occur, especially in the fingers. The body thus regulates the body temperature.

The primary form of Raynaud’s phenomenon usually manifests itself for the first time in or just after puberty. The primary form of Raynaud’s typically affects women under the age of 30 (usually in the teenage years or early twenties).

In the secondary form of Raynaud there is, besides having the primary form, also an underlying reason and getting Raynaud’s phenomenon can be the result of a rheumatic disease with deviations in the vessel walls.

The secondary form of Raynaud’s phenomenon is “secondary” to another condition. The onset is later in life, usually after the age of 30. The conditions are numerous and can include several autoimmune diseases. The most common rheumatic diseases associated with Raynaud’s include scleroderma and lupus erythematosus, but can include others such as rheumatoid arthritis, inflammatory myositis and Sjögren’s syndrome.

Cause of Raynaud’s phenomenon

Raynaud’s phenomenon is caused by overly-sensitive blood vessels within the fingers, toes and nose. They are more affected by cold and stress. The blood vessels narrow substantially, causing a decrease in blood flow and associated color change. The lack of blood flow often results in a pale or white discoloration. Subsequently, the digits can turn blue and eventually red as there is a sudden influx of blood into the digits after the episode is over.

Primary form of the disease
An attack of Raynaud’s phenomenon can be provoked by stimuli from the outside or from within the body. In the primary form of Raynaud’s phenomenon these are:
• too rapid a transition from hot to cold, for example in hand washing.
• touching a very cold object.
• experience violent emotions, such as fear, sadness or anger.
• working with vibrating objects.
The primary form does not arise as a result of another illness.
In the primary form of Raynaud’s a great sensitivity to stimuli as a temperature change (a rapid transition from hot to cold, a cold environment or the touch of a cold object), smoking, fluid or a violent emotion (fear, grief or anger) is already enough to provoke an attack. By external stimuli, caused by working with vibrating devices (such as chainsaw, drilling machine), striking rhythmic keys (such as playing piano) or working on a keyboard, such an attack can also be provoked.

How does the body react in the fingers or toes?
The body responds with the temporary shutdown of the blood supply to the fingers and toes, so that in case of an attack the blood vessels in your fingers or toes become so narrow that they allow little or no blood to pass through. Vascular cramps and discoloration from white to blue-purple usually occur simultaneously with both hands, but the thumbs do not change. During the discoloration your hands and feet can feel cold and numb and hurt. After some time the blood supply starts again and the skin becomes warm and red or purple again; the fingers start to tingle, swell and hurt.

Hereditary factors or hormones?
It is not certain whether Raynaud’s phenomenon is hereditary, even though it occurs more often in certain families than in others.
Presumably there is a connection between the female sex hormone and Raynaud’s phenomenon. In pregnancy, the symptoms are less and during the ovulation the hands of women are extra sensitive. The contraceptive pill has little or no effect on Raynaud’s complaints.

Secondary form of the disease
In the secondary form there is an underlying reason. If this is the case, Raynaud’s phenomenon brings on more problems. Sometimes inflammation of the blood vessels of the fingers or toes leads to the formation of a blood clot, which can cause painful ulcers or even death of tissue.
In a number of patients with initially an apparently primary form of Raynaud’s, a systemic disease develops within a few years. Rheumatic conditions, which can cause Raynaud’s phenomenon, are:
rheumatoid arthritis (RA)
Sjögren’s syndrome
systemic lupus erythematosus (SLE)
• polymyositis
• dermatomyositis
giant cell arteritis.
In addition, arteriosclerosis and certain drugs can also cause this secondary form.

Damage to the capillaries in the hand, fingers or toes
In the secondary form of Raynaud’s phenomenon there is often permanent damage to the capillaries in the hand, fingers or toes. These damages can give a reduced blood supply and with low stimuli from outside the blood supply can decrease even further. An attack can take five minutes to half an hour. The fingers or toes become white and ‘numb‘ due to the reduction of blood flow and they turn blue-purple when all oxygen is out. During the discoloration your hands and feet can feel cold and the feeling is reduced. After the attack, the blood vessel relaxes, it widens and there is a lot of oxygen-rich blood in the finger or toe; that gives the red color and the tingling. Sometimes the toes or fingers swell and they are painful. Eventually the symptoms disappear again.
Inflammation of the blood vessels of the fingers or toes also occurs, which can sometimes lead to the formation of a blood clot, which can cause painful ulcers or wounds or even death of tissue.

Difference in attacks between primary and secondary forms?
The attacks in the secondary form are similar to those in the primary form. The difference is that in the secondary form the course of the disease depends on the underlying condition and the attacks do not occur on both hands or feet at the same time.

Complaints with Raynaud’s phenomenon

Raynaud’s phenomenon occurs when there is a decrease in blood flow to different parts of the body, such as the fingers and toes. The fingers or toes typically change colors varying from white, blue and red. This can be associated with a “pins and needles” sensation (numbness) or discomfort. These symptoms occur intermittently and are known as “episodes” or “attacks” and tend to resolve on their own.

Primary form of Raynaud’s phenomenon
In the primary form of Raynaud, we see vascular cramps and discolouration of the fingers or in other parts of the body occur with external or internal stimuli. Internal stimuli are, for example, emotions like fear, fright, surprise, grief, annoyance, excitement. External stimuli are, for example, a cold environment, rapid temperature changes and drafts, touching cold objects, handling vibrating equipment and playing rhythmic tests such as typing or playing the piano.
Location complaints. In the primary form, the vascular cramps and discoloration usually occur on both hands at the same time and not in the thumbs. The symptoms also occur in other parts of the body, such as the toes, the nose tip, the earlobes or the tongue.
Age. The primary form is most common, sometimes at a young age and can naturally diminish as you grow older.
Severity disorder. The primary form is usually not serious but difficult. In severe forms of the primary form, the fingers, toes, ears or nose may feel cold, swear or turn black in case of very severe attacks.

Secondary form of Raynaud’s phenomenon
The secondary form of Raynaud’s phenomenon can arise as a result of various underlying diseases. The attacks occur under the same conditions as in Raynaud’s primary form, but in the secondary form of Raynaud’s phenomenon there are often permanent deviations of the capillaries. In the secondary form of Raynaud, the symptoms often do not occur simultaneously on both hands or feet.
Underlying disease. It may take years before an underlying disease is found. Usually this is a rheumatic disease that goes along with deviations in the vessel walls, for example a chronic inflammation of the connective tissue.
Blood vessels. In other cases, the explanation for the secondary form of Raynaud is very clear: the blood vessels in the fingers have suffered damage through prolonged work with vibrating hand tools, on a keyboard or by playing the piano. If the side of the hand is used as a hammer, it can also cause damage (hypothenar hammer syndrome). If blood circulation is not good, wounds heal more slowly and reduce nail growth. The fingers are in daily use constantly in soft and uncomfortable touch with their environment. Normally, small skin lesions heal quickly. In case of a disturbed blood supply this can take a long time or you may develop sores. Characteristic of the secondary form of Raynaud are ulcers on the fingertips, along the nails or on the toes. In the worst case, in 10% of patients, the tip of a finger or toe can die off.
Side-effects of certain drugs. The secondary form of Raynaud can also arise as a side-effect of certain drugs, such as beta-blockers (which are prescribed for high blood pressure and heart disease) and for example ergotamine (for migraines).

Difference between primary and secondary form.
The secondary form of Raynaud is more severe than Raynaud’s primary form, depending on the underlying vascular disease or vascular damage. It is possible that the symptoms start as the primary form of Raynaud and pass on to the secondary form of Raynaud. One person is more sensitive to this than the other.

Diagnosis Raynaud’s phenomenon

Your doctor will base your diagnosis on a combination of the medical history, his findings and additional test results.

At the first visit, the doctor asks you a number of questions to get a good picture of your condition. You can think of the following questions:
– When did your complaints start?
– Can you describe your complaints?
– How are your working conditions?
– What medication do you use?
– Do rheumatic diseases occur in your family?
– What is your medical history?
– Are you under the treatment of other specialists and for what?

Raynaud’s phenomenon is diagnosed based on the medical history and physical examination.

Physical examination
The doctor will be looking for:
• signs of connective tissue diseases and blood vessel inflammations
• joint complaints and muscle weakness
• dry mouth and eyes
• skin rash and skin changes
• fever
• swallowing disorders
• anxiety

Blood test
Patients with primary Raynaud’s typically have a normal examination and blood work. Looking at the blood vessels below the fingernails (nailfold capillaroscopy) is used to help differentiate primary from secondary Raynaud’s. On an X-ray picture the narrowed arteries in the hand and fingers and the capillaries (very small fine blood vessels) under the nailfold can be seen.
An autoimmune serological examination may be necessary, for example to see if you have certain anti-nuclear antibodies (ANA) in your blood or a capillary microscopy if the doctor suspects a systemic disease or a developing systemic disease. Patients with secondary Raynaud’s typically have an abnormal exam and/or blood work.

Treatment of Raynaud’s phenomenon

Drug treatment
Drugs without a prescription
• For the pain you can get a simple painkiller with the active ingredient paracetamol without a prescription. Paracetamol helps against pain and fever, does not cause stomach problems, usually does not cause side effects and can be easily combined with other medications.
• An NSAID, an anti-inflammatory analgesic in a lower dose. The abbreviation NSAID stands for Non-Steroidal Anti-Inflammatory Drugs. These painkillers inhibit inflammation.

Do you have physical complaints? Always go to your doctor or specialist for a proper diagnosis and proper treatment.

Drugs on prescription
This is provided by your doctor or rheumatologist or internist.
Many patients are able to control their symptoms with a lifestyle modification alone. But sometimes drugs are needed, also because of underlying diseases and many options are available.

Diseases, which can cause the second form of Raynaud’s phenomenon, are:
* rheumatoid arthritis (RA)
* Sjögren’s syndrome
* scleroderma
* systemic lupus erythematosus (SLE)
* polymyositis
* dermatomyositis
* giant cell arteritis.

Treatment by vasodilating drugs
Providing vasodilating drugs or ointments that dilate the cramped blood vessels. Vasodilators are blood pressure medications that open (dilate) blood vessels. They affect the muscles in the walls of your arteries and veins, preventing the muscles from tightening and the walls of the blood vessels from narrowing. Vasodilating drugs have a relaxing effect on the blood vessels, so the blood flows better. But they can have unpleasant side-effects such as headaches, palpitations, dizziness, flushing and swollen feet.
A widely used vasodilating drug is a so-called calcium channel blocker. Calcium channel blockers (amlodipine, nifedipine, felodipine and others) and angiotensin-receptor blockers dilate the contracted blood vessels. These medications act by increasing blood flow to the fingers and toes. This will reduce the number, severity and duration of the attacks of painful, cold fingers and toes. Alpha blockers are also prescribed. Alpha blockers dilate the contracted blood vessels and reduce the number, severity and duration of the seizures. Sometimes blood thinning can also help. However, if you stop taking these drugs, Raynaud’s symptoms return.

For patients with more severe symptoms or who have developed complications such as ulcers on the fingertips, other medications can be used including sildenafil or prostacyclins.

Sometimes, patients will also be prescribed other medications that can help improve symptoms, including topical creams, selective-serotonin-reuptake inhibitors (SSRIs) or cholesterol-lowering (statin) medications.

Treatment by nerves
• The temporary switching off of the nerve by an infusion with a vasodilating and anti-inflammatory drug. If you have serious complaints and have not been given enough medication and ointments, you can get an infusion in the hospital with a vasodilator and anti-inflammatory drug to make your blood more fluid. This promotes blood flow so that wounds (for example on the fingers) heal faster.
Cutting through a nerve (nerve block), so that the muscles in the vascular wall do not cramp anymore. In very serious cases of Raynaud’s phenomenon, it can be decided to completely eliminate a nerve (sympathectomy). It concerns the nerve that runs through the chest cavity to the fingers or the nerve that runs through the abdominal cavity to the toes. Sometimes an anesthesiologist can first locally anesthetize the nerves to investigate whether the surgery will correct the problem.
You can opt for the temporarily working chemical sympathectomy or the long-term or permanent thoracoscopic sympathectomy. In the chemical sympathectomy, the anaesthesiologist gives you an anesthetic injection in your neck. The injection is easy to repeat, but you can get a drooping eyelid (‘Horner’) from this procedure. In the thoracoscopic sympathectomy, a tube is placed in your chest. The surgeon then searches for the nerve via a ‘viewing operation‘ and cuts it through. The result is a warm, dry hand and arm. You will no longer perspire there. The effect of surgical sympathectomy surgery on these nerves lasts longer than a chemical sympathectomy. The effect on the nerve in the chest cavity can last up to about two and a half years. You have a chance of various unpleasant side effects: dry hands, a drooping eyelid and excessive sweating on the trunk. By an operation on the nerve in the abdominal cavity, Raynaud’s phenomenon on the toes sometimes remains permanent. But here, too, you are more likely to have serious side-effects, such as impotence and malfunctions in the function of the bladder.

Treatment by massage
Giving a connective tissue massage. The massage improves circulation and relaxes the blood vessels.

Use of drugs and adherence

• It is important that you take the drugs as prescribed. In order to get your illness under control, it is very important that you take your drugs on a regular basis every day. If you do not, there is no good concentration of the drug in your blood and therefore the drug will work less well.
• Do you have trouble remembering when and how many of your drugs you should take on a daily basis and at what time of the day? Then buy a drug dispenser, in which you can sort your drugs per day.
• Do you have difficulty or objection to taking the drug prescribed to you, for example because of possible side-effects? Then that can lead to you no longer taking the drug regularly. Discuss this with your doctor!

Deterioration of your illness and/or complications
The doctor will look at:
• The dosage and amount of drugs you use.
• Switching to another drug in the same type or a completely new type of drug.
• Combining different drugs at the same time.

Your doctor can tell you
• whether the drug can work for you.
• how you can best use the drug.
• how much and how often you can use the drug.
• how you can best reduce the drug.

You should always tell your doctor or specialist
• if you use other drugs (bought or prescribed by another doctor).
• if you have another medical condition.
• whether you will be operated on soon.
• if you have previously been prescribed an anti-inflammatory analgesic that has caused side effects.
• whether you want to become pregnant or are pregnant.
• if you are breast-feeding.
This is important because your doctor or specialist has to make a careful choice between different drugs.

• All drugs may have side-effects when used, so ask your doctor or pharmacist what side-effects you can expect or read the leaflet.

Reduce or stop
• If you stop using a drug yourself or reduce the number of drugs yourself, your symptoms may worsen. Always consult your doctor or specialist first, if you want to use less drugs or want to stop.

Alternative treatments

There are many types of alternative treatments. This refers to all treatments that fall outside normal scientific medical care. No scientific evidence has been provided for the operation of these treatments. The alternative treatments are also called ‘complementary‘ because they can supplement the regular medical treatment by your doctor.
Many people choose an alternative treatment in addition to their regular medical treatment. They hope that this helps extra against their complaints or better helps to deal with the complaints.

Can an alternative treatment be a replacement for your regular medical treatment?
No, if you opt for an alternative treatment method, it is always a supplement to your regular medical treatment. You should not stop your regular treatment, because otherwise you run unnecessary health risks. Always consult your attending doctor before you start an alternative treatment.

What alternative treatments are there for example?
There are many different alternative treatment methods available, which can also be combined. Some examples are:
• Chinese medicine (acupuncture, pressure point massage, nutrition and tai chi).
• homeopathy (products made from plants and minerals).
• Bowen therapy.
• Ayurveda.
• Bach flower treatment.
• shiatsu, foot-sole and classic massage.
• Touch for Health.
• Reiki.
• dietary supplements.

What do you have to pay attention to?
• Many alternative treatments have not shown that they actually work. If you choose to try alternative treatment, make sure that your complaints do not increase. Stop an alternative treatment as soon as your symptoms increase.
• Always consider first why you want to follow an alternative treatment.
• Prepare yourself by looking up and reading information about the alternative treatment. Consult with your attending doctor, because certain complaints may be reduced by some alternative treatment methods.
• Determine yourself in which alternative method you trust for the relief of your complaints and whether you want to start.

What can you do best when choosing an alternative treatment?
• Always consult your doctor about the alternative treatment method you want to follow.
• Consult with your attending doctor and with the alternative therapist if they want to discuss your treatment with each other.
• Choose an alternative therapist who has followed a recognized vocational training and is affiliated to a professional organization.
• Ask your alternative therapist in advance about the purpose, duration, costs and risks of the treatment. How much money you spend depends on which treatment you choose and how long it takes.
• Do not stop your regular medical treatment, as this may worsen your symptoms.
• Weigh during treatment whether you want to continue or stop if your symptoms get worse, if you do not notice any effect of the alternative treatment or if you get side-effects.

Why is your food important?

• Healthy nutrition is important to get the vitamins and minerals and other nutrients that your body needs.
• Obesity creates risks for your health and disease progression. In the case of arthritis and obesity, for example, obesity plays an important role in overburdening and osteoarthritis in the knees, hips and ankles. The pressure on your joints is then simply too great. Healthy eating and exercise (exercising a sport and active in the home and outdoors) can help to reduce or prevent excess weight.
• A healthy diet is always important and the dietitian can support you with the right dietary advice.

What is a healthy diet?
With a healthy diet your body gets the right amount of good nutrients, which it needs. You eat the quantities that your body needs. The dietitian can support you with the right nutritional advice.
The 5 courses to choose from are:
• Vegetables and fruit
• Bread, cereal products and potatoes
• Fish, legumes, meat, egg, nuts and dairy
• Lubrication and preparation fats
• Drinks.

Where can you get advice for a healthy diet?
• You can request advice from your doctor or a dietitian. The doctor can refer you to a dietitian. Some diets may possibly help against your symptoms. Consult with your doctor or dietitian if you want to try a certain diet. And make sure that you do not omit important foods from your daily diet.

What can you do with a painful or dry mouth?
Sometimes you can suffer from a dry or painful mouth for various reasons, also due to your illness. Tips:
• by chewing (for example piece of cucumber, sugar-free candy or chewing gum) and sucking (ice cube) the salivary glands are stimulated to produce saliva.
• good oral care is important: good tooth brushing, flossing and the use of mouthwash.
• rinse your mouth regularly, drink small amounts of water and use a mouth sprayer if necessary.
• let hot drinks cool down first.
• use ice or cold dishes, because the cold numbs the pain.
• do not use sharp herbs and spices, fruit juice, carbonated soft drinks, alcoholic beverages, very salty foods and sour food.
• food with hard crusts, nuts, bones and bones can cause injuries.
• use soup, gravy or sauce with the hot meal to make the food smoother.
• spreadable cheese, spreadable paté or salad, jam or honey on bread, porridge, drinking breakfast and custard, for example, swallows easier than dry spreads.
• if you need to use ground or liquid food, you can use a mixer to grind your meal with some extra moisture.

In some forms of arthritis or use of certain drugs intestinal complaints can occur. The intestinal complaints can arise from the influence of certain drugs on food intake. Conversely, the food you eat can have an effect on the way your drugs work.

Dietary supplements, fish fatty acids, glucosamine and vitamin D?
• Dietary supplements are available as pills, powders, drops, capsules or drinks and are intended as a supplement to inadequate daily nutrition. They contain vitamins, minerals or bio-active substances. These synthetic or isolated vitamins, minerals or bio-active substances have the same effect as the vitamins and minerals that are already naturally in your food and drink.
Many people choose an additional supplement, in addition to their daily diet. The users of dietary supplements indicate that they experience positive effects of the use of certain herbs, vitamins and minerals. The body absorbs the nutrients in pills more easily than the nutrients in food. But if you eat healthy and varied, you do not need additional nutritional supplements, because you already get enough nutrients, minerals and vitamins. Always report to your doctor and pharmacist that you are using dietary supplements.
• Fish fatty acids appear to have a mild anti-inflammatory effect with a high intake. Research has been done especially in rheumatoid arthritis. The advice is to eat oily fish twice a week.
• Glucosamine can act as a mild painkiller for osteoarthritis in the knee, but does not stop arthritis.
• Our body naturally makes vitamin D under the influence of outdoor sunlight. Vitamin D may have a beneficial effect in inflammatory arthritis, but that has not yet been proven sufficiently. People with Lupus erythematosus get vitamin D prescribed as they are not often outside, because sun exposure can lead to lupus flares and skin problems. Vitamine D is given with calcium tablets to ensure a good intake in the body, when you have osteoporosis or osteoarthritis.

Living with Raynaud’s phenomenon

Avoid big temperature changes. Ensure that no doors or windows are open in the house, as drafts can provoke vascular cramps. If you have a bath, use it, because the heat of a shower is much less even dosed. Always get into a warm bed with a jug or an electric blanket. Warm your home evenly, a central heating is the best option. Wear warm clothes in the house and wear an extra sweater when you go to colder rooms.
Take good care of your skin. If it is cold outside, keep the skin on your face, your hands and feet smooth with an ointment.
Wounds on fingertips and toes heal more difficult due to the lack of a normal amount of oxygen because of poor blood circulation. You can keep a deep wound clean by rinsing it daily under a lukewarm shower. Then pat the wound dry with a clean cotton towel and cover it with a bandage that does not stick to the skin. On top of that you can put a kind of cap over the fingertip. Do not use an ointment on the wound because an infection can develop under the ointment.
Drink less alcohol and eat healthy. Limit alcohol consumption to a maximum of two glasses a day for men and one glass a day for women. Ensure a healthy weight, if you are too skinny, you will have a shortage of subcutaneous adipose tissue and in order to withstand the cold the body also needs food as fuel.
Keep moving. Movement improves the flow of blood in your body.
Do not smoke. If you smoke, you will get nor-epinephrine in your blood, this substance causes the blood vessels to contract. In addition, the temperature in your arms and legs drop 2 or 3 degrees Celsius with every cigarette, cigar or pipe. Only after 24 hours that temperature is back to normal again.
Learn how to deal with emotions and stress. Adrenaline is a substance that causes the blood vessels to contract and comes free when you experience fear, fright, surprise, sadness, annoyance and excitement. Chronic stress also results in an increased adrenaline level. So try to go through life as relaxed as possible. Give yourself peace, learn to set limits and try to find out which factors trigger an attack.
If you do get an attack, warm your hands by putting them in your sleeves, under your armpits or between your legs.
Be careful with hot water and (central) heating. It is better to gradually warm up your hands. The feeling in your hands can be numbed and you can hurt yourself more easily.
Be careful with certain drugs. Please consult your doctor on this!
Certain drugs for migraines, high blood pressure or heart disease can narrow the blood vessels. In particular, beta-blockers and ergotamine can cause the secondary form of Raynaud’s phenomenon in some people. There are more drugs that can have a negative effect, especially cold remedies with ephedrine.
Wear appropriate clothing to keep your body warm. Wear more layers of clothing over each other, that is better for the insulation and the absorption of perspiration. Wear gloves and warm socks. If it is cold outside, first warm your socks and gloves indoors.
Ensure a good working environment. A profession where you have to be outside, work in a cold or draft room or where you have to sit for a long time may be less suitable for you. This may mean that you have to discuss your complaints not only with your own doctor, but also with your employer and the health and safety service. In case of wet chores in the household, wear rubber gloves and avoid cold water. Put on gloves when you take something out of the freezer. Watch out carrying heavy shopping bags, the handles pinch the blood flow to your fingers. Use a shopping cart, a backpack, a shoulder bag or a bicycle bag.

Demystifying Medicine I Exploring Raynaud’s Phenomenon

Video source
Demystifying Medicine I Exploring Raynaud’s Phenomenon I https://youtu.be/beXQZBJC6Lc

Accountability text Raynaud’s Phenomenon
The information about Raynaud’s Phenomenon is general.
Every situation is different, so if you have any questions or complaints, always consult your doctor, medical specialist or pharmacist.

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