Raynaud’s syndrome is a seizure-like reduction in blood flow to the acras (outermost extremities) as a result of vasoconstriction (vascular spasms), which affects women about four to five times more often than men. Depending on the particular form of the disease, Raynaud’s syndrome is easily treatable and has a good course.
What is Raynaud’s syndrome?
Raynaud’s syndrome is the term used to describe seizure-like ischemic states (reduced blood flow) caused by vasoconstrictions or vasospasms (vascular spasms), which primarily affect the arteries of the fingers and toes. The attacks are characterized by ischemia (pallor due to reduced blood flow), in the further course cyanosis (bluish skin changes) as well as painful reactive hyperemia (excessive blood accumulation) due to the irritation of vessel sections. As Raynaud’s syndrome progresses, it is also accompanied by damage to the vessel walls such as a thickening of the intima (innermost layer of the vessel wall) and/or a capillary aneurysm (vessel dilation) with subsequent necrosis (cell death) and gangrene (self-dissolution of dead tissue). In principle, two forms of Raynaud’s syndrome are differentiated. While primary Raynaud’s syndrome cannot be attributed to an underlying disease, secondary Raynaud’s syndrome is associated with certain diseases such as thrombangiitis (chronic inflammatory vascular disease), scleroderma (connective tissue disease), and trauma (e.g., working with jackhammers) or intoxications (e.g., heavy metals, certain medications).
Causes
The causes of primary Raynaud’s syndrome have not yet been conclusively determined. Presumably, a genetic predisposition to vasospastic disease underlies the condition, as in many cases it can be associated with other conditions caused by vasospasm, such as migraine or angina pectoris (Prinzmetal’s angina). Involvement of hormonal factors is also discussed for the manifestation of primary Raynaud’s syndrome. Secondary Raynaud’s syndrome, on the other hand, is due to various diseases that cause Raynaud’s syndrome to manifest. About 40 underlying diseases (including thrombangiitis obliterans, collagenoses, rheumatoid arthritis, arteriosclerosis, cryoglobulinemia, cold agglutinin syndrome) are known to cause secondary Raynaud’s syndrome. In addition, certain medications (beta blockers, ergotamine), excessive drug and nicotine use, and poisoning with heavy metals or vinyl chloride can cause secondary Raynaud’s syndrome.
Symptoms, complaints, and signs
Raynaud’s syndrome presents with attacks of cold or (emotional) stress. During such an attack, discoloration of the skin is symptomatic, occurring primarily in the hands and feet. In rare cases, the nose, ears, forehead and chin are also affected. In pregnant women, in very rare cases, the nipples may also be affected. Most commonly, it is a white discoloration or blue discoloration (cyanosis) of the skin. In other cases, a biphasic discoloration may be observed, from a white discoloration to a blue discoloration. The discoloration of the skin can also be observed as a tricolor phenomenon, in which first a white discoloration, then a blue discoloration and finally a red discoloration occurs. The red discoloration is explained by increased blood flow to the skin (hyperemia), which is usually accompanied by a tingling or tapping sensation. The Raynaud’s attack can last from a few minutes to an hour. Pain or a tingling sensation may occur during the staining. The colorings may occur symmetrically (primary Raynaud’s syndrome), seen here mainly in the index finger, middle finger, ring finger and little finger, or asymmetrically (secondary Raynaud’s syndrome). In a few cases, the circulatory disturbances can lead to secondary damage, for example, necrosis of the skin, i.e., the death of individual cells.
Diagnosis and course
Raynaud’s syndrome can usually be diagnosed on the basis of characteristic symptoms such as pain and discoloration due to reduced blood flow. Here, a symmetrical pattern of affection of the second to fifth fingers points to a primary Raynaud’s syndrome. If an attack is provoked by the application of cold (ice water), relief can be achieved by the application of heat or vasodilator nitroglycerin.If, on the other hand, there is an asymmetric pattern of involvement of individual fingers and no reduction in symptoms can be achieved by the addition of heat and/or nitroglycerin, it is more likely to be a secondary Raynaud’s syndrome. In addition, oscillography can be used to check the blood flow to the affected fingers or toes, while Doppler sonography allows conclusions to be drawn about occlusions or constrictions in the vessels. The course and prognosis of primary Raynaud’s syndrome are very good. In secondary Raynaud’s syndrome, the course depends largely on the underlying disease.
Complications
In a severe course, the end limbs of the toes and fingers are no longer supplied with sufficient oxygen. As a result, the tissue may become diseased or even die. As a result, the fingertips or even entire fingers must be amputated. If the cramps persist over a longer period of time, this also leads to vascular damage in the long term. If the disease occurs as a result of scleroderma, there is also a risk of permanent changes in the blood vessels. Here, too, the affected tissue must be amputated. If the symptoms are severe, drug therapy is required, which is always associated with complications. If glycerol trinitrate is prescribed, the so-called “nitrate headache” may occur. With initial use, there is also a risk of a drop in blood pressure, increase in pulse, dizziness and feelings of weakness. Occasionally, allergic skin reactions occur or the patient suffers a collapse. A severe complication is peeling rash. This severe skin reaction occurs in isolated cases and often leads to loss of hair and nails. If infusion – such as with prostaglandins – is necessary, this may be associated with infection, injury, and occasionally thrombosis.
When should you see a doctor?
Because Raynaud’s syndrome is an incurable disease, a doctor must always be consulted for this condition. There is no self-healing, and the symptoms can only be treated purely symptomatically and not causally. The earlier the treatment of the disease is initiated, the higher the probability of a positive course of the disease. A doctor should be consulted if the affected person suffers from discoloration of the skin due to Raynaud’s syndrome. These occur mainly in strong stress situations, although they can also be triggered by heat or cold. The skin may also turn blue. If these symptoms occur over a longer period of time, a doctor must be consulted. As a rule, disorders of blood circulation also indicate Raynaud’s syndrome and should always be examined by a doctor. Raynaud’s syndrome can be examined and treated by a general practitioner or by a dermatologist.
Treatment and therapy
Therapeutic measures for Raynaud’s syndrome can only aim to reduce the presenting symptoms, as the condition itself cannot be cured. Physical measures, medications to be applied systemically and topically (locally), as well as acupuncture and alternative healing methods are used for Raynaud’s syndrome. In addition to education on prophylactic measures such as early wearing of gloves or use of pocket warmers to avoid cold exposure, which would worsen the symptoms of primary Raynaud’s syndrome, training of the finger muscles is recommended. In addition, if the patient is taking medications that may cause Raynaud’s syndrome (beta-blockers, certain antidepressants, hormones, ergotamines), a change in medication should be considered. ACE inhibitors and Ca antagonists (calcium antagonists such as felodipine, nifedipine, amlodipine) are administered as part of the drug therapy of Raynaud’s syndrome, especially in the case of already frequent severe attacks, whereby patients with seasonal attacks are recommended to abstain from medication if they are free of symptoms. In addition, nitroglycerin can be applied topically for severe seizures to relieve symptoms. In advanced stages of secondary Raynaud’s syndrome, prostaglandins (iloprost, alprostadil) can be infused intravenously to prevent or treat necrosis. In addition, one study demonstrated that classical Chinese acupuncture techniques can lead to significant improvements in the severity and frequency of attacks in primary Raynaud’s syndrome.
Prevention
Because the causes of primary Raynaud’s syndrome are unknown, no direct preventive measures exist. However, certain factors that may trigger manifestation or new attacks should be avoided. These factors include, for example, avoidance of cold exposure, nicotine abstinence, and a reduction in work with highly vibrating work equipment (e.g., jackhammers). Early and consistent treatment of the underlying condition may prevent secondary Raynaud’s syndrome.
Follow-up
Regular check-ups once a year with the attending physician or in a specialized outpatient clinic are mandatory. Wounds must be treated immediately by a competent person to ensure that they heal well. Conscientious follow-up is essential, especially for new-onset wound healing disorders. Patients who suffer from Raynaud’s syndrome or have struggled with it in the past should conscientiously follow all recommended precautions. Ideally, this will prevent the recurrence of symptoms. Aftercare also includes continuing to eat as healthily as possible. Caffeine-containing stimulants should be used sparingly. A diet rich in vitamins, on the other hand, can protect the blood vessels. At the same time, it is important to refrain from smoking and avoid smoky rooms. The same applies to stress. Exercise is beneficial to recovery because it stimulates blood flow. It is advisable to take painkillers and cold remedies only after consulting a doctor who is informed about the patient’s medical history, as these remedies may contain substances that constrict the blood vessels. Affected persons should always wear warm gloves in cold weather and it is better not to attack cold objects without protection. If, despite all precautions and aftercare measures, a Raynaud’s attack occurs again: as an immediate measure, hold your hands under warm water or in the armpits, massage and move them. In this way, the vessels quickly dilate again.
This is what you can do yourself
In Raynaud’s syndrome, the first thing to do is to avoid the triggers. The hands and fingers must be particularly protected from cold and wet. In winter, it is important to always wear well-insulating gloves, for example, with a neoprene layer. Only heated gloves or pocket warmers may help. Pressure on the palm of the hand increases the symptoms. This occurs, for example, when riding a bicycle or working with the hands in cold weather. If such triggers cannot be avoided, preventive measures such as finger gymnastics, movement of the arms and hands, and regular pressure relief will help. Vasoconstrictive substances should be avoided. This includes nicotine in particular, but also some medications. Informing the doctor about the disease is therefore extremely important, even if the current symptoms appear to have nothing to do with Raynaud’s syndrome. Overall, a healthy lifestyle also improves vascular health. Reducing stress and restlessness, as well as a balanced diet rich in vitamins, have a protective effect and can delay the progression of the disease. Exercise with endurance training is particularly important, as it optimizes blood flow throughout the body and has a calming effect on the nervous system. All of the above measures cannot cure the disease, but they can improve the quality of life and delay its progression.