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Raynaud's Syndrome

Raynaud's syndrome: description

Raynaud's syndrome is a vascular disease caused by vasospasm. The cramps usually occur in attacks on the fingers, less often on the toes and other parts of the body. This reduces the blood supply to the affected body region - they become pale and cold, which is why one speaks of a cadaver finger or white finger disease. The cramps are usually triggered by cold and psychological stress.

The syndrome was first described in 1862 by the medical student Maurice Raynaud. Doctors today differentiate between two forms of Raynaud's syndrome: The cause of the first is unknown (primary Raynaud's syndrome). The second form occurs in the context of other diseases (secondary Raynaud's syndrome), for example in scleroderma (a hereditary disease of the connective tissue), after injuries or through overdosing on certain drugs.

Primary Raynaud's syndrome is mainly found in women between the ages of 20 and 40. Overall, women are about five times more likely to be affected than men. An estimated three percent of the population have the typical symptoms of primary Raynaud's syndrome.

Raynaud's syndrome: symptoms

Typical of Raynaud's syndrome is that fingers (with the exception of the thumb) or the toes turn pale and later blue due to the disturbed blood supply. Those affected suffer from abnormal sensations, numbness and sometimes pain. The attacks usually do not last longer than half an hour, but can also last longer. Subsequently, the skin is often reddened. These symptoms of Raynaud's syndrome are known as tricolor phenomena.

In primary Raynaud's syndrome, symptoms appear on both hands or feet. In secondary Raynaud's syndrome, usually only one side is affected.

If the cramps (spasms) persist over a longer period of time, the vessels are permanently damaged. Then the tissue can die - necroses form. However, such damage usually only occurs as complications of secondary Raynaud's syndrome.

If Raynaud's syndrome is the result of scleroderma (hereditary disease of the connective tissue), the skin of the hands, arms or face is also thickened and tense. In addition, the blood vessels in the affected area are changed.

Raynaud's syndrome: causes and risk factors

Raynaud's syndrome is caused by very strong and sudden narrowing of the blood vessels (especially in the fingers and hands), which loosens again after a while. This is known as vasospasm. The seizures occur particularly in cold temperatures and under stress. The cause of this phenomenon is an imbalance of vasodilating and vasoconstricting factors.

The vascular spasms in Raynaud's syndrome can be caused by disorders of the blood vessels in the toes and fingers or by impaired nerve activity. Disorders in the hormonal balance can also play a role. How these various factors interact is still unclear.

The causes of primary Raynaud's syndrome are still largely unknown. Young women are particularly affected. In the course of life, the attacks usually become increasingly rare and weaker. Primary Raynaud's syndrome is often more common in the family. Above all, smoking promotes the development of Raynaud's syndrome.

Secondary Raynaud's syndrome can be triggered by a variety of different diseases. These include rheumatological diseases, but also diseases of the nerves (e.g. multiple sclerosis) or vascular diseases such as arteriosclerosis. Cancer diseases, especially of the blood-forming system, can also cause Raynaud's syndrome.

Sometimes a carpal tunnel syndrome, in which certain nerve tracts in the wrist are pinched, goes hand in hand with Raynaud's syndrome. Scleroderma, an autoimmune disease that leads to hardening of the connective tissue in the skin, often results in Raynaud's syndrome. In the context of autoimmune diseases, so-called cold agglutinins can sometimes be detected in the blood. Cold agglutinins are antibodies that clump together when it is cold. The body reacts to this with inflammatory reactions, which in turn promote vasoconstriction and thus promote Raynaud's syndrome.

Some medications (contraceptives, cytostatics, interferon, beta blockers, ergotamine preparations and dopaminergic substances) or drugs (cocaine, designer drugs) can also cause Renauld syndrome. Some people who come into contact with certain chemicals in their job (e.g. polyvinyl chloride) or who work with vibrating machines such as jackhammers or chainsaws for a long time also develop Raynaud's syndrome.

Raynaud's syndrome: examinations and diagnosis

The first point of contact with Raynaud's syndrome is your family doctor, who may refer you to a rheumatologist. As a rule, a detailed description of the symptoms is sufficient to make a diagnosis of "Raynaud's syndrome".

The medical consultation provides important information on the type and cause of Raynaud's syndrome. During the conversation, the doctor will ask the following questions, among other things:

  • Do you notice any sudden discolouration of your hands, possibly associated with pain?
  • Do the symptoms appear symmetrically on both hands?
  • Do the symptoms often occur under stress or in the cold?
  • Have you noticed any changes in your hands?
  • Do you have any known previous illnesses?
  • Do you know of similar cases in your family?

A series of tests can help diagnose Raynaud's syndrome. With the help of the fist test, for example, the influx of blood into the fingers can be checked by comparing the sides. To do this, the doctor tightly clasps the patient's wrist and lets him close and open his fist around 20 times. If Raynaud's syndrome is present, the fingers will usually be bass in this experiment.

The Allen test is used to examine the arteries that carry blood to the hand. The doctor squeezes one of the two arteries one after the other (radial artery or ulnar artery) and checks whether the respective open artery is supplying the hand with sufficient blood. If the hand becomes pale during compression, the uncompressed artery is probably blocked.

A cold provocation test can be used to determine whether cold can trigger an attack. To do this, the hands are dipped in ice water for around three minutes. However, this investigation is controversial because the attacks cannot be reliably triggered in this way.

If you suspect Raynaud's syndrome, it is also important to inspect your hands. The doctor pays attention to wounds and tissue damage such as dead areas on the fingertips, so-called rat bite or fingertip necrosis. The doctor also looks for changes to the nail.

Diagnosis of primary Raynaud's syndrome

The criteria for diagnosing primary Raynaud's syndrome are:

  • Both hands are affected.
  • The attacks mainly occur when it is cold or stressful.
  • There is tissue damage.
  • Symptoms have been present for over two years without an underlying disease being diagnosed.
  • Further examinations are normal.

Another reason for a primary form of Raynaud's syndrome is if those affected are young (under 30) and female, or if they also suffer from migraines or a special form of heart disease (Prinzmetal's angina) - both diseases are based on spasms of certain blood vessels.

Diagnosis of secondary Raynaud's syndrome

The criteria that speak for the presence of secondary Raynaud's syndrome are:

  • Only one hand is affected.
  • The tissue in the affected regions is damaged.

If the symptoms occur in men who are over 30 years old, this also speaks more for secondary Raynaud's syndrome. Certain illnesses, for example kidney inflammation or bone pain, as well as the use of certain medications or the consumption of drugs, increase the suspicion of secondary Raynaud's syndrome.

A number of further examinations are then possible for the final clarification.

Capillary microscopy

As part of a capillary microscopy, the doctor examines the smallest vessels (capillaries) in the hands. This can be used to determine, for example, scleroderma as the cause of secondary Raynaud's syndrome. This disease is associated with giant capillaries, vascular-free regions, and minor bleeding.

Collagenoses - fight against your own connective tissue

  • Skin lupus (cutaneous lupus erythematosus)

    As the name of this collagenosis suggests: cutaneous lupus is most noticeable on the skin. Areas that are exposed to direct sunlight, such as the face and hands, are particularly affected. Reddish patches of skin appear over the nose and cheeks. Because of its shape, doctors speak of butterfly erythema. In addition, there is a nodular and blotchy rash, enlarged veins, damage to the mucous membrane in the mouth and nose or ring-shaped / oval plaques with scales. They can scar in chronic lupus.
  • Systemic lupus erythematosus

    In systemic lupus, various organs can become inflamed. Affected are, for example, the lungs, heart muscle and sac, kidney, liver or eyes. But the skin is not spared either: as in skin lupus, it is very sensitive to light. Those affected often complain of joint and muscle pain. In addition, defects in the mucous membrane can occur in the mouth. In addition, patients suffer from general symptoms such as fever, fatigue, night sweats or weight loss.
  • Dermato- and polymyositis

    Weak muscles and paralysis? These are typical signs of polymyositis. The shoulder and pelvic muscles are often affected. Muscle pain, swelling of the gums, swallowing and speech disorders can also occur. With dermatomyositis there are also symptoms on the skin: a red-bluish rash on the face ("purple disease") and redness and swelling around the eyes. Redness, flaking and small nodules (Gottron papules) appear on the hands. Internal organs such as the heart, lungs or esophagus can also become inflamed.
  • Systemic sclerosis (scleroderma) and Raynaud's syndrome

    Doctors distinguish between two forms: the more easily progressing limited systemic sclerosis (later organ involvement, less pronounced) and the rapidly progressing diffuse systemic sclerosis. An early symptom of systemic sclerosis is Raynaud's syndrome, which occurs in the hands and has three stages: first the fingers become pale, then they turn blue (lack of blood circulation) and then, with pain, red (return of blood circulation).
  • Systemic sclerosis of the skin

    People with scleroderma often feel exhausted and sad. Many are sensitive to the cold. Joints are also difficult to move. In the further course the skin changes: Hardening due to calcium deposits, Madonna fingers (waxy, claw-like bent fingers), a mask-like facial expression, a tobacco pouch mouth, enlarged veins and skin discoloration (hypo- and hyperpigmentation).
  • Systemic sclerosis in the body

    If the disease spreads to organs, in most cases the gastrointestinal tract is affected, which can no longer move properly due to the sclerosis. This causes difficulty swallowing, gas, constipation and cramping abdominal pain. The lungs are also affected in many cases (pulmonary fibrosis, pulmonary hypertension). In diffuse scleroderma, in particular, the tissue in the kidneys, heart, salivary glands and joints can become inflamed and thus change.
  • CREST syndrome

    One form of scleroderma is so vivid that doctors have created their own acronym for it: CREST syndrome. The individual letters stand for the symptoms: C - Calcinosis cutis with skin knots on the fingers and areas exposed to pressure, some with tissue defects; R - Raynaud's Syndrome; E - Esophageal dysmotility: severely restricted mobility of the esophagus with difficulty swallowing and heartburn; S - sclerodactyly with skin changes typical of scleroderma on the fingers; T - telangiectasia.
  • Sjogren's syndrome

    In Sjogren's syndrome, it is mainly salivary and lacrimal glands that become inflamed, but also those that produce vaginal secretions. This also explains the symptoms of those affected, such as a dry mouth, swollen salivary glands, dry eyes or a dry vagina with pain during sex. Experts also summarize the symptoms of dryness as the so-called sicca syndrome. In addition, typical rheumatic complaints such as joint pain, tiredness or sadness can occur with Sjogren's syndrome. Inflammations in organs or vessels are also possible.
  • Mixed collagenosis

    Mixed collagenosis, also known as Sharp syndrome, is a clinical picture that consists of several types of collagenosis (typically lupus, scleroderma, polymyositis) and rheumatoid arthritis. Its symptoms are correspondingly diverse. But they are quite mild here. Those affected always suffer from Raynaud's syndrome.
  • A visit to the doctor brings clarity

    If you notice any signs of any of the above-mentioned collagenoses, it is best to see a doctor as soon as possible. Your family doctor is a good first point of contact here. He can classify your complaints and initiate further investigations. He also prescribes the first medication, e.g. If the suspicion is confirmed, he will also refer you to a specialist. Even if the disease is a lifelong companion, early action can slow the progression of the disease.
  • A visit to the doctor brings clarity

    If you notice any signs of any of the above-mentioned collagenoses, it is best to see a doctor as soon as possible. Your family doctor is a good first point of contact here. He can classify your complaints and initiate further investigations. He also prescribes the first medication, e.g. If the suspicion is confirmed, he will also refer you to a specialist. Even if the disease is a lifelong companion, early action can slow the progression of the disease.

Blood tests

Blood tests can reveal other conditions that lead to secondary Raynaud's syndrome. Important parameters to be ascertained are the blood count, platelets, inflammation parameters and so-called protein and immune electrophoresis. In addition, certain antibodies should be determined. These include ANA and anti-DNA antibodies, which are typical of the rare immune disease lupus erythematosus. This can also cause secondary Raynaud's syndrome.

Imaging procedures

Magnetic resonance angiography and duplex sonography enable the detection of vascular cramps (spasms), constrictions (stenoses) and other vascular changes. It can be important to also examine the heart and neck vessels. A so-called α-blocker can be given during the examination. This medication causes the constriction to open. If this is not the case, a vascular disease can be assumed.

It is important to rule out other diseases that are associated with symptoms similar to Raynaud's syndrome. These include embolisms and peripheral arterial occlusive disease (PAD), in which blood vessels become blocked. In addition, the so-called isolated acrocyanosis is accompanied by a painless blue coloration of the hands. A discomfort at first glance, but harmless, is spontaneous bruising on the finger (the so-called spontaneous finger hematoma).

Read more about the examinations

Find out here which examinations can be useful for this disease:

Raynaud's syndrome: treatment

Raynaud's syndrome therapy is initially based on general measures. It is crucial to avoid the triggers of the attacks, especially stress and cold. Avoiding drinks and food that are too cold can also reduce the frequency of attacks. Those affected benefit from heated gloves. People with Raynaud's syndrome should also stop smoking. Some people also benefit from a diet rich in omega-3 fatty acids.

Stress relief

A major contribution to improving symptoms is reducing stress. Learning a relaxation technique such as autogenic training or progressive muscle relaxation is helpful. Exercise also helps reduce stress.

Wound care

Wounds in the affected areas must be treated intensively and professionally, as they may heal poorly and thus persist for a long time.

What to do in the event of a Raynaud attack?

If an attack is imminent, hands should be washed with warm water. It is also advisable to move and massage your hands so that the vessels expand again quickly. Sometimes it also helps to put your hands under your armpits.

A Raynaud attack usually subsides on its own after a maximum of half an hour - before permanent damage occurs. However, if the vascular constriction does not loosen, bed rest and warmth often help. If that is not enough either, the doctor can prescribe anticoagulant therapy, for example with heparin, possibly in combination with other drugs.

Medication

If the general measures are not sufficient, it is possible to take medication against Raynaud's syndrome. The administration of medication is particularly useful if the tissue is damaged and a good blood circulation has to be ensured. The most important group of drugs against Raynaud's syndrome are calcium channel blockers, for example nifedipine. In addition, nitroglycerin, a vasodilator substance, can be applied to the affected areas.

However, the drugs can have undesirable side effects. The calcium channel blockers can make the fingers swell, a nitro ointment can cause headaches.

In the case of very severe Raynaud's syndrome, a number of other drug groups can be prescribed, some of which are not specifically approved for Raynaud's syndrome therapy. The use of these drugs to treat Raynaud's syndrome, but especially antidepressants, is controversial.

If secondary Raynaud's syndrome is caused by narrowed vessels, therapy with acetylsalicylic acid (ASA) and a cholesterol-lowering drug (statin) can be considered.

Operations

In particularly severe cases, an operation can be useful. This includes, for example, the blockage of vasoconstricting nerves (sympathectomy). Such a blockage can also take place with medication with the active ingredient guanethidine. This only works for a limited time, but it can be enough to allow wounds and tissue damage to heal.

Unless that Raynaud's Syndrome occurs in connection with work, a job or even a change of occupation may be necessary.

Read more about the therapies

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Raynaud's syndrome: disease course and prognosis

Primary Raynaud's syndrome is bothersome and uncomfortable, but it is harmless and usually only slightly restricts the quality of life. The symptoms usually get better over time.

In secondary Raynaud's syndrome, the pressure of suffering can be significantly higher and intensify over time. The course of the disease varies greatly from case to case due to the many different causes. Some patients experience complications such as poorly healing wounds or other tissue damage, which can be complicated and lengthy to treat. In particularly severe cases, areas of tissue can also die. However, since new vessels form relatively quickly in the event of vascular damage, an amputation, for example of the affected finger, is necessary Raynaud's Syndrome only very rarely necessary.

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