Raynaud's disease. Causes, symptoms, diagnosis and treatment of pathology

Raynaud's disease is a disease caused by a lesion of the human autonomic nervous system, which is manifested by trophic disorders and pain, mainly localized in the fingers. To date, the causes of Raynaud's disease have not been studied enough, but it has been proven that women suffer from this disease 5 times more often than men. Factors contributing to the development of the disease are hereditary predisposition and constitutional deficiency of the vasomotor innervation of the terminal vessels.

Raynaud's disease symptoms

Patients with Raynaud's syndrome note the appearance of a feeling of tingling, burning in the fingertips, blue or whitening of them that occurs after smoking, exposure to cold, or during exercise.

Do not confuse the disease and Raynaud's syndrome. In the latter case, the symptoms of the disease appear as a result of another pathology (vibration disease, diencephalitis, rheumatism, scleroderma, etc.) and may disappear on their own when it is cured.

During the course of the disease, 3 main stages are distinguished:

1. The angiospastic stage, which is characterized by short-term spasms of the vessels of the terminal phalanges of the fingers, they become cold to the touch and turn pale, while patients complain of numbness of the fingers. The spasm passes quickly, the skin on the phalanges of the fingers acquires the same color and temperature. Attacks can be triggered by smoking, hypothermia of hands (even washing with cold water), physical exertion on the fingers (playing the piano, guitar, sports activities).
2. The angioparalytic stage may develop after the first stage, but in some cases the disease begins with it. During this period, patients periodically develop cyanosis of the skin of the terminal phalanges, which can persist for several hours. This stage is characterized by the appearance of pain and burning sensation in the fingertips. During attacks of cyanosis, small blisters may appear on the skin, which, bursting, heal over time, but small scars remain in their place.
3. The trophoparalytic stage is characterized by the appearance of severe trophic disorders in the phalanges of the fingers. Non-healing ulcers appear on the skin, which subsequently necrotic. In the most severe cases, gangrene of the fingers develops, and the process may affect the osteoarticular apparatus of the fingers.

Sometimes, some patients may have different stages of Raynaud's disease on different fingers. It should be noted that the disease can affect not only the fingers, but also the legs.

Raynaud's disease treatment

Treatment of Raynaud's disease is usually long-term, and includes a whole range of measures. First of all, it is necessary to identify and eliminate the factors that lead to the onset of seizures. Patients need a complete diet with enough vitamins and minerals. It is also recommended to observe the regime of work and rest, and sometimes a change of residence or work.

Drug therapy is aimed at relieving pain, improving tissue nutrition, preventing ischemia attacks in the terminal phalanges of the fingers, and, if necessary, treating purulent complications.

Of the drugs in the treatment of the disease, the following groups of drugs are used:

• antispasmodics (papaverine hydrochloride, no-shpa);
• painkillers (analgin, ibuprofen, ketorol);
• vasodilators (corinfar, diltiazem);
• anti-inflammatory drugs (acetylsalicylic acid);
• local antibacterial drugs (levomekol, baneocin) and systemic action;
• vitamin complexes (biomax, complivit).

The use of acupuncture and physiotherapy increases the effectiveness of treatment. Patients with Raynaud's disease are shown the following types of physiotherapy treatment:

• local darsonvalization;
• ultraviolet irradiation of the upper cervical and lumbar segments of the spine;
• electrophoresis with calcium or lidase;
• radiotherapy;
• diathermy;
• galvanizing baths.

Drug treatment of the disease should be prescribed only by a doctor, since the drugs used in treatment regimens have a direct effect on other organs and systems of the body. Conservative treatment of Raynaud's disease can last for several years, until it loses its effectiveness. After that, to alleviate the condition of patients, doctors have to resort to surgical methods of treatment.

Patients undergo a sympathectomy operation, the essence of which is to remove the nerve fibers that cause spasm of the arteries, provoking attacks of the disease. The operation can be performed endoscopically, which reduces the risk of complications and reduces the period of rehabilitation after surgery.

Recently, information has appeared about the development of a fundamentally new method of treating Raynaud's disease using stem cells. The developers of the method claim that in most cases, stem cell therapy can completely stop the progression of the disease or greatly alleviate its course.

Which doctor to contact

Medical treatment of Raynaud's disease includes taking antispasmodics, painkillers, drugs that improve microcirculation, vitamin and mineral complexes.

If Raynaud's disease is suspected, it is necessary to contact an angiologist, and if it is impossible to do this, a rheumatologist should be consulted. Additionally, you will need to consult a cardiologist and a vascular surgeon. In the diagnosis of Raynaud's disease, dopplerography of the affected arteries is important.

Raynaud's disease is a pathological condition known to medicine since 1862. It is based on a paroxysmal spasm of the blood vessels of the peripheral parts of the extremities, the face. Spasm occurs, for example, in response to exposure to cold, vibration or severe stress.

As a result, a person feels pain at the site of the spasm, numbness, a feeling of crawling appear. The affected area becomes white at first and then turns blue. The skin becomes cold to the touch. When the attack ends, the skin turns red, and in this area there is a feeling of heat. With prolonged existence of the disease, trophic disorders develop.

Raynaud's disease should be distinguished from Raynaud's syndrome, since despite the similarity of symptoms, they differ in etiological factor. The fact is that after Maurice Raynaud described the signs and etiology of the disease, it was found that it can develop as an independent disease due to impaired functioning of the central nervous system, and can act as a syndrome of some other pathologies. This is the reason for the difference between the two concepts.

Causes

The causes of Raynaud's disease cannot be considered separately from the mechanism of the development of the disease. It is based on violations of the organic and functional plan, affecting both the vascular walls and the apparatus responsible for their innervation. As a result, there is a violation of the nervous regulation of blood vessels, so they react to various effects with spasms, followed by increasing atrophy.

Causes of Raynaud's syndrome:

1. Blood viscosity disorders: cryoglobulinemia, polycythemia vera, Waldenström's macroglobulinemia.
2. Osteochondrosis of the cervical upper thoracic region.
3. Prolonged exposure to vibration during the development of vibration disease.
4. Lack of autonomic nervous regulation - syringomyelia.
5. Autoimmune diseases affecting connective tissue: systemic lupus erythematosus, systemic scleroderma, rheumatoid nodosa, rheumatism, Sjögren's syndrome, dermatomyositis, periarteritis.
6. Vascular diseases are Takasyau disease, obliterating atherosclerosis of the legs, etc.
7. Peripheral nerve lesions on the background (polyneuropathy).
8. Intoxication of the body with lead, arsenic salts, cytostatics and ergotamine.
9. Violations in the functioning of the adrenal glands, thyroid and parathyroid glands.
10. Rarely, Rein's syndrome provokes accessory cervical rib syndrome, carpal tunnel syndrome, and scalenus anterior syndrome.

In turn, the causes of Raynaud's disease lie in the pathologies of the central nervous system and spinal cord with the involvement of the hypothalamus, brain stem and cortex in this process. These pathological processes lead to the fact that the impulses that regulate the work of blood vessels are transmitted with violations.

First signs

Raynaud's disease manifests itself as follows:

• in humans, the fingers of the limbs turn pale;
• there is a sensation of tingling;
• limbs go numb;
• the patient's blood flow to the fingers is disturbed, they become bluish;
• there is pain in the limbs;
• the temperature rises;
• blood pressure rises;
• headaches and dizziness are observed;
• there are difficulties with the coordination of limb movements.

With warming and emotional stability, the symptoms do not immediately go away, the limbs become red. These symptoms can also affect not only the limbs, but also the auricles, the tips of the lips, the tip of the nose. Raynaud's disease is often accompanied by severe migraines. If these signs appear, it is urgent to contact a specialist who will diagnose and recommend adequate treatment for the disease.

Symptoms and stages

The main symptoms of Raynaud's disease in women and men are characterized by a predominant lesion of the fingers (more often - the index, middle and ring fingers, less often - the thumb and little finger), manifested by a gradual change in the state of the blood vessels and tissues of the affected area. The severity of these manifestations is determined by the stage of the disease and the duration of its course. Much less commonly, other exposed areas of the body (toes, chin, tips of the ears and nose) are affected, which are also regularly exposed to cold or heat and other risk factors.

In the clinical picture of Raynaud's syndrome, there are:

• the first stage;
• second stage;
• third stage.

The first stage of Raynaud's syndrome

The first stage is also called angiospastic, which is due to the nature of the damage to the blood vessels (that is, their spasm, narrowing). At this stage of development, the first symptoms of the disease appear, which are of a short duration.

The first stage of Raynaud's syndrome can be manifested by the following symptoms:

1. Pain. The occurrence of pain is also due to a violation of blood circulation in the fingers. The fact is that under normal conditions, the cells of the body constantly emit by-products of their vital activity (lactic acid and others), which are carried away with the blood flow to the place of neutralization in other organs. With spasm of arterioles, blood circulation is disturbed, as a result of which metabolic by-products accumulate in the area of ​​\u200b\u200bthe formation and lead to the appearance of pain (pain in this case can be burning, stabbing, aching). After normalization of blood circulation, toxic substances accumulated in high concentrations are carried with the blood flow to nearby tissues, which can lead to a short-term increase in pain in the phase of reflex vasodilation.
2. Change in skin color. It is the first manifestation of the disease. The first occurrence of this symptom is associated with a violation of the nervous and hormonal regulation of vascular tone, which is always due to the action of provoking or predisposing factors. As a result of a sharp spasm of small blood vessels (arterioles), the blood supply to the vessels decreases. Since the pinkish color of the skin is due to the presence of blood in the capillaries, with vasospasm, the skin of the affected area becomes pale in color. A characteristic feature of Raynaud's syndrome is a clear limitation of the lesion from unaffected areas (it may seem to a person as if his fingers were dipped in paint). The vasospasm lasts for a rather short period of time (2-4 minutes), after which their reflex expansion occurs, the vessels overflow with blood and the skin becomes bright red. At this stage, edema may appear in the joints, which is due to increased permeability of the vascular walls and sweating of the liquid part of the blood into the surrounding tissues. Within 10-30 minutes, the vascular tone returns to normal, the skin acquires its normal color and all clinical manifestations disappear.
3. Numbness of fingers. Numbness in the affected area occurs as a result of impaired delivery of blood and nutrients to nerve fibers, which are extremely sensitive to hypoxia (lack of oxygen). At the beginning of the attack, the patient may feel a slight tingling sensation or “crawling on the skin” (these symptoms are typical for the initial stage of nerve damage), but after a few minutes the sensitivity is significantly reduced until it disappears completely.
4. Decreased tissue temperature in the affected area. A constant body temperature is maintained by blood circulation, which heats up passing through the internal organs (liver, muscles), and cools in the area of ​​the skin and other peripheral tissues. With a spasm of blood vessels, blood stops flowing into the area of ​​​​the fingers, as a result of which the skin quickly cools down (local temperature can decrease by 2 - 4 degrees or more).

The second stage of Raynaud's syndrome

The second stage develops approximately six months after the onset of the first symptoms of the disease and is characterized by a more pronounced violation of the mechanisms of regulation of vascular tone. Characterized by a decrease in the frequency and increase in the duration of seizures, which may occur as a result of exposure to provoking factors or spontaneously.

A distinctive feature of this stage of the disease is the pronounced cyanosis of the fingertips, which occurs after vasospasm. This is explained by the fact that under normal conditions, the oxygen contained in the blood cells (erythrocytes) is transferred to the cells of various tissues, which in return release carbon dioxide (a by-product of cellular respiration). Oxygenated blood (arterial) is red, while venous blood (saturated with carbon dioxide) is bluish in color. Normally, carbon dioxide released by cells is quickly carried away with the bloodstream to the lungs, where it is released with exhaled air. However, in conditions of impaired blood circulation, the concentration of carbon dioxide in the blood increases significantly, as a result of which the skin becomes bluish.

It is also worth noting that the second stage of Raynaud's disease is characterized by a pathological expansion of the venous vessels, which further enhances the manifestations of the disease. After a long vasospasm, pain is usually more intense and prolonged than in the first stage.

The third stage of Raynaud's syndrome

It develops 1-3 years after the onset of the disease and is characterized by irreversible damage to the tissues of the fingers associated with circulatory disorders. Attacks of spasm of blood vessels with their subsequent expansion can have a different frequency and intensity, accompanied by severe pain.

The third stage of Raynaud's disease is characterized by the following complications:

1. infectious complications. The circulating blood contains immune cells that protect the body from infection. If local blood circulation is disturbed, the risk of developing infectious diseases of the skin and soft tissues of the fingers increases, which is also facilitated by local ischemia and necrosis.
2. The necrosis (necrosis) of soft tissues. Due to insufficient blood supply to tissues, cell death occurs in the most affected areas (the skin of the fingertips). Dead tissue is shed over time, and sores appear in their place. They can reach several millimeters in depth, rarely bleed, and are painless. Healing of ulcers takes a long time (days, weeks) and leads to the formation of dense scars.

Raynaud's disease: photo

What Raynaud's disease looks like, the photo shows the hands of a woman with this disease:

Diagnostics

Which doctor should I contact if I suspect this disease? If Raynaud's disease is suspected, it is necessary to contact an angiologist, and if it is impossible to do this, a rheumatologist should be consulted. Additionally, you will need to consult a cardiologist and a vascular surgeon.

The first diagnostic criterion for Raynaud's disease is a persistent vasospasm of the skin: when warmed, blood circulation is not restored, the extremities remain cold and pale.

In the study of patients with Raynaud's disease, it should first of all be established whether the phenomenon is not a constitutional feature of the peripheral circulation, that is, a normal physiological reaction under the influence of cold of varying intensity.

Laboratory studies are required:

• general blood analysis;
• on total and c-reactive protein, albumin and globulin fractions;
• detailed coagulogram, fibrinogen level, properties of platelets and erythrocytes.

Recently, experts have noted the high efficiency of a new method for diagnosing Raynaud's disease - wide-field capillaroscopy of the nail bed. This method has a high accuracy of diagnosing the disease.

The final diagnosis of Raynaud's disease can only be established as a result of a thorough examination. If no other diseases are identified that caused the occurrence of the symptom complex, the diagnosis of "Raynaud's disease" is established.

How to treat Raynaud's disease?

With Raynaud's syndrome, disability is issued mainly in connection with the main disease (rheumatism, scleroderma, etc.). But sometimes, if the patient cannot perform work related to his profession, then disability is also possible in connection with Raynaud's syndrome II or III stage.

Persons with stage III Raynaud's syndrome are unsuitable for military service, with stage II - limited fit, with stage I - subject to conscription.

The provision of emergency assistance in case of an attack is:

1. Elimination of the factor that provoked the attack
2. Warming the diseased area - massaging with a woolen cloth, taking a hot drink
3. Reception or injection of vasodilators and analgesics, antispasmodics (drotaverine, no-shpa, platifillin).

With Raynaud's disease, treatment in women and men is long-term. First of all, it is aimed at treating the underlying disease, which caused the occurrence of the symptom complex.

You should stop smoking and avoid exposure to provoking factors at work and at home - contact with cold air and cold water, exposure to vibration, prolonged work on a computer keyboard and heavy metal products, contact with various industrial chemicals, psychological stress.

The drugs are prescribed:

• vasodilating action (antagonists and calcium channel blockers) - nifedipine (Corinfar, Cordipin, Cordaflex, Calcigrad, Nifedipine, Nifecard, Osmo-adalat, Fenigidin), nicardipine, verapamil (Isoptin, Finoptin, Verogalid)
• ACE inhibitors - Captopril, Kapoten
• serotonin receptor blockers - ketanserin
• prostaglandins - Vazaprostan, Vap, Caverject, Alprostan
• improving the physical and chemical properties of blood and microcirculation - Agapurin, Trental, Dipyridamole, Pentoxifylline, Vasonite

Drug treatment must be combined with physiotherapy and non-traditional treatments. Physiotherapy - UHF, mud therapy, hyperbaric oxygen therapy, galvanic baths, physiotherapy exercises, reflexology. With the ineffectiveness of the ongoing medical and physiotherapeutic treatment, surgical intervention is possible - sympathectomy. One of the modern methods of treating Raynaud's syndrome is stem cell therapy, which contributes to the normalization of peripheral blood flow.

Physiotherapy

Treatment methods are aimed at relieving symptoms, and during remission - at stopping seizures. The methods are quite effective, especially in the initial stages of the disease and are very diverse.

Lymphatic drainage:

• massage of the collar zone - this activates the regional blood supply and reflex zones. Massage enhances microcirculation and improves lymph flow, preventing edema from appearing. The course includes at least 15 procedures;
• magnetotherapy - a traveling magnetic field reduces the tone of the venules, which improves the outflow of blood and lymph. The procedure is carried out at different magnetic frequencies, at least 10 times;
• segmental vacuum therapy - the cervico-thoracic region or the collar zone is affected using a vacuum applicator. At the same time, the hydrostatic pressure gradient increases, which leads to a decrease in the tone of arterioles and an active outflow of lymph.

Vasodilating methods:

• electrophoresis of vasodilators - for example, with nicotinic acid, which quickly relieves swelling and reduces painful symptoms;
• ultratonotherapy is a combination of high voltage currents and supersonic frequency. Increases the outflow of blood and lymph;
• barotherapy - exposure to high and low pressure, especially indicated for obliterating endarteritis;
• IR irradiation - stimulates capillary blood circulation and improves the supply of soft tissues with oxygen.

Hypocoagulant methods:

• low-frequency magnetotherapy - exposure to a low-frequency field. Improves blood supply to tissues and trophism;
• laser irradiation of blood - the effectiveness of the procedures is based on the absorption of a laser beam of a certain length by the molecular structures of the blood. Thus, the rheological composition of the blood improves, which leads to a decrease in spasmodic phenomena.

They also resort to stimulating procedures: pearl shower, thalassotherapy, aerotherapy, contrast baths.

An unconventional approach to treatment

First of all, patients diagnosed with Raynaud's syndrome are recommended to massage the affected areas - fingers, hands, lower limbs. Massaging movements must begin with the fingertips, gradually moving to the shoulder. In this case, the movements should be smooth - you can stroke the skin, rub, pinch, pat. Such a massage should be carried out for at least 2 weeks for 10 minutes. After that, you need to take a break for 1 week, and then repeat the course again. If the clinical symptoms of the disease extend to the earlobes, then they also need to be massaged, stroked and rubbed.

To make the treatment even more effective, massage can be carried out by first moistening the hands with massage oil with the addition of a few drops of mint, anise, motherwort or yarrow essential oil. These oils have antispasmodic and analgesic effects.

Hot baths have proven themselves well with the addition of a decoction of herbs to the water - motherwort, valerian root, dill inflorescences, yarrow. You can also add a few drops of the essential oils listed above to the water. The duration of the therapeutic bath is 15 minutes, while the water temperature should not exceed 39-40 degrees. During this time, the patient warms up properly, the blood supply to small blood vessels increases, and the heartbeat quickens.

Instead of a bath, you can try baths. They are prepared in the same way as a therapeutic bath, only hands or feet are dipped in water. It is important to monitor the temperature of the water - you can not keep the limbs in the bath after the water has cooled. Not only will this not be beneficial, but it can also exacerbate Raynaud's attacks.

Lifestyle

Medication treatment is a factor from which it is impossible to "get out". No matter how much a person wants to stop taking medication, he should not do this so that the disorder does not become even more severe. In addition, he should not take them occasionally, but according to a strict system, as prescribed by the doctor. Treatment carried out in any way will not lead to any result.

As already mentioned, physical procedures are also needed. Without them, drug treatment will not have a good effect. However, the correct lifestyle of the patient plays an important role. It is he who will allow as little as possible to experience attacks of spasms. What should a person suffering from Raynaud's disease do, and what should he not do?

1. You definitely need to quit smoking. Nicotine contributes to severe vasospasm, especially in people who already suffer from problems with blood vessels.
2. The patient should avoid any vibrating devices. For example, you can not hold a working coffee grinder in your hands, touch a food processor, work with an electric drill and other equipment that creates vibration. Even a vacuum cleaner can contribute to the occurrence of another attack. It is not always possible to evade such work, because a person cannot completely do without any household appliances. In such cases, he is advised to use them as little as possible and be sure to wear woolen gloves to reduce vibration.
3. You should always keep your limbs warm. Even if signs of the disease are observed only on the hands, there is no guarantee that they will not appear on the legs after constant hypothermia. Therefore, mittens should be warm, and shoes should be dry and also warm. The patient should not wash their hands with cold water. If hot water is not always supplied to the apartment, it is better to heat it up on the stove and wash your hands with warm water, this will definitely not cause another spasm.
4. The most important factors influencing the occurrence of seizures are nervous shaking and overwork. Therefore, we must avoid these factors and strive for peace and peace of mind.

By following these rules, a person may not be afraid of the constant manifestation of the Raynaud phenomenon. This is the name of the form of the course of an attack of the disease, consisting of three phases:

1. Paleness and decrease in temperature of the fingers with sensations of pain;
2. The appearance of cyanosis and increased pain;
3. Subsidence of pain and the return of normal skin color or redness.

Operation

There is also a surgical treatment of Raynaud's syndrome, which is indicated in case of resistance of the disease to complex medical and physiotherapeutic treatment. The essence of the procedure lies in the denervation of the vessels that feed the affected areas. This intervention is called sympathectomy. In this case, with the help of a scalpel, nerve fibers are cut, along which an impulse passes, causing vasospasm. The expediency of this method of treatment is considered only in severe cases of Raynaud's syndrome.

It is worth noting that among doctors there is a different point of view on this method of treatment. The fact is that in some cases, a few months after surgical treatment, the symptoms of the disease return again, and therefore this method of treatment is not considered effective. And, of course, one cannot do without the help of a surgeon when trophic disorders lead to gangrene.

Prevention

Prevention of the disease is to keep the limbs always warm.

To do this, it is necessary to wear layered clothing, especially to protect the hands and feet. Mittens are more practical than gloves, as they keep fingers warm. Special electrically heated gloves and socks and hand warmers are now commercially available. Some people manage to prevent attacks of Raynaud's disease by making quick circular swings with their hands: under the influence of centrifugal force, blood is pumped into the limbs. Warm water helps keep you warm, but you need to make sure that it is not too hot. People with Raynaud's disease should not smoke.

Raynaud's disease can be prevented by avoiding triggers and starting treatment at the first sign of it. But in severe cases, surgery may be required. Raynaud's disease is an early symptom of other diseases, such as systemic sclerosis, characterized by thickening of the skin. However, Raynaud's disease should not be considered a general sign of its onset.

Treatment prognosis

Raynaud's disease is not completely curable. A person suffering from this disease is forced to follow the recommendations described above all his life and periodically resort to physiotherapy methods. Depending on the severity of the disease, drug therapy can be both permanent and prescribed courses.

If Raynaud's syndrome is diagnosed, and the underlying disease is curable, then it is quite possible to get rid of vascular spasms. As a rule, recovery leads to the disappearance of the factors that provoke the syndrome.

Among the vegetative-vascular diseases of the distal extremities, Raynaud's disease occupies a leading position. Information about her is quite contradictory. As a rule, the disease does not pose a threat to life, however, it limits the possibilities and requires constant drug therapy.

For citation: Alekperov R.T., Starovoitova M.N. Raynaud's syndrome in the practice of a therapist // BC. 2010. No. 27. S. 1695

Raynaud's syndrome (SR) is an episode of transient digital ischemia due to vasoconstriction of digital arteries, precapillary arterioles, and cutaneous arteriovenous shunts under the influence of cold temperature and emotional stress.

The syndrome was first described by Maurice Raynaud in 1862 as "local asphyxia of the limbs", and Tho-mas Lewis further introduced its division into primary Raynaud's disease and secondary Raynaud's phenomenon.
Epidemiology
The prevalence of SR, according to the results of epidemiological studies, ranges from 2.1 to 16.8% in different countries. Differences in the prevalence of SR obtained in individual studies are partly explained by the fact that in different studies the results were obtained using mail or telephone surveys, questionnaires distributed randomly among the population, or the inclusion in the study of only those who sought medical advice about subjective sensations, accompanying SR. In Europe, the highest frequency is in the UK and France, and the lowest in Spain and Italy. These data confirm the well-known position that in countries with a relatively cold climate, the frequency of SR is significantly higher than in countries with a warmer climate. The peak incidence occurs in the 2-3 decades of life. Women predominate among patients with SR and, according to different authors, the ratio of women to men ranges from 2:1 to 8:1. The age of onset of SR in women is significantly less than in men. With age, there is a trend towards an increase in the proportion of men among those with SR. So, in one of the studies, the ratio of men / women under the age of 50 was 1:5.2, and in the older age group - 1:1.1.
Pathogenesis
Despite the fact that almost 150 years have passed since the first description, the pathophysiology of Raynaud's syndrome remains not completely clear and, apparently, SR is a multifactorial disease. It is believed that the cause of excessively pronounced vasospasm in response to provoking stimuli is a defect in the central and local mechanisms of regulation of vascular tone. Among these factors, vascular endothelial, intravascular and neuronal disorders are important. In secondary SR, structural changes in the vessels are also an important factor. A key role in the genesis of SR is played by an imbalance between vasodilators and vasoconstrictors due to disturbances in the neurogenic control of vascular tone and mediators produced in the hemocirculation. Among vascular mediators, great importance is attached to changes in the production of nitric oxide, endothelin-1, serotonin, thromboxane and angiotensin, the level of which in patients with SR increases under the influence of cold. Furthermore, the regulation of vascular tone depends not only on humoral vasodilating mediators, but also on neuropeptides released from the respective nerve endings. Disturbances in the balance of a number of neuronal mediators, including calcitonin gene-associated peptide, neuropeptide Y, and agents interacting with α-adrenergic receptors also lead to insufficient vasodilation and increased vasoconstriction. Along with this, the activation of platelets to oxidative stress is important.
Classification
SR is divided into primary (idiopathic, or Raynaud's disease) and secondary, associated with other diseases. To verify the variant, diagnostic criteria for primary and secondary SR based on clinical features, data from laboratory and instrumental studies were proposed (Table 1). In most cases, SR is idiopathic, accounting for 80-90% of the total number of patients.
It should be noted that the antinuclear factor (ANF) has a relatively low prognostic value for connective tissue diseases (30%), while the detection of specific autoantibodies significantly increases the likelihood of secondary SR. Approximately 15-20% of patients with SR who have specific autoantibodies and/or capillaroscopic changes, but no symptoms of connective tissue diseases, later (usually within two years) develop one or another connective tissue disease. The clinical significance of SR is due to its high prevalence in the population and frequent association with other, often life-threatening patients, diseases (Table 2). Secondary SR is most often associated with systemic scleroderma, systemic lupus erythematosus, other connective tissue diseases, hematological disorders, and certain medications.
Clinic
Factors that provoke vasospastic reactions, which are also called Raynaud's attacks, are low ambient temperature and / or emotional stress. Clinically, SR is manifested by clearly delimited areas of sequentially changed color of the skin of the fingers: pale blue-red (the so-called three-phase SR). The first two phases of color change reflect the state of vasospasm and hypoxia, and at the end of the attack of vasospasm, due to reactive hyperemia, the skin acquires a bright red color. Classical three-phase Raynaud's attacks are noted only in 15% of patients, while in most cases (in 85%) two-phase color changes are observed. A significant proportion of patients complain of sensory disturbances (numbness, tingling, pain) during Raynaud's attack.
Raynaud's syndrome has some clinical features:
. Most often, color changes are observed on the fingers of the hands.
. Changes begin on one finger, then spread to other fingers and become symmetrical on both hands.
. The II-IV fingers of the hands are most often involved, the thumb usually remains intact.
. Changes in skin color can also be noted in other areas: auricles, tip of the nose, face, above the knees.
. During Raynaud's attacks, livedo reticularis may appear on the limbs, which disappears after the completion of vasospasm.
. In rare cases, there is a lesion of the tongue, which is manifested by its numbness and transient speech disorders (becomes slurred, blurry).
Vasospasm usually lasts 15-20 minutes and ends with a rapid restoration of blood flow, as evidenced by the intense pink color of the skin (reactive hyperemia). At the same time, the frequency and duration of episodes of vasospasm can vary both in different patients and in the same patients at different times of the year (more intense in winter than in summer).
Diagnostics
SR is established primarily on the basis of complaints and clinical symptoms of the disease. The diagnosis of SR is considered possible with a positive answer to the following three questions:
- Is there an unusual sensitivity of the fingers to cold?
- Does the color of the fingers change when exposed to cold?
- Do they become white and/or bluish?
At the same time, it should be taken into account that the sensitivity of the fingers to cold is also noted among absolutely healthy people. So, in a survey of approximately 7 thousand people, almost 12% of them answered in the affirmative to the question: “Are your fingers or limbs unusually sensitive to cold temperatures?” . In addition, cold skin or ill-defined mottling of the skin of the fingers, hands, and extremities is considered a normal response to cold exposure. Such a sign as whitening of the fingers is highly sensitive for SR (94-100%) and has a high specificity (75-78%).
The British Scleroderma Research Group has proposed the following definition of CP:
. Reliable SR - repeated episodes of biphasic skin discoloration in the cold.
. Probable SR is a single-phase discoloration of the skin, accompanied by numbness or paresthesia under the influence of cold.
. No SR - there are no changes in skin color under the influence of cold.
In all patients with newly diagnosed SR, it is necessary to conduct special studies with the aim of differential diagnosis of the primary and secondary nature of the pathology.
First of all, you need to clarify:
. whether the patient has symptoms of connective tissue diseases with which SR is most often associated (arthritis, myalgia, fever, "dry" syndrome, skin rash, cardiopulmonary disorders);
. whether the patient is taking any drugs at the time of the study, especially chemotherapeutic agents;
. whether the patient is exposed to vibration or other mechanical influences that injure the hands;
. whether episodes of Raynaud's syndrome are associated with certain positional changes.
Along with the diagnosis of SR as such, it is equally important to determine the clinical form. Primary SR in most cases are moderate in nature, and only in 12% of patients the symptoms of the disease are significantly pronounced. The median age of onset of primary SR is 14 years and only 27% of cases develop at age 40 or older. In 1/4 of patients, SR occurs in first-line relatives.
Despite the identity of the clinical manifestations of primary and secondary SR, in these two conditions there are some differences in individual signs. The following signs indicate the likelihood of a secondary nature of SR:
. late age of onset
. male,
. painful episodes of vasospasm with signs of tissue ischemia (ulceration),
. asymmetric attacks.
It should be taken into account that clinical signs of SR-associated disease may develop several months or years after the onset of SR. Instrumental and laboratory research methods are aimed primarily at clarifying the nature of Raynaud's syndrome - primary or secondary. The most informative among instrumental methods is nail bed capillaroscopy (CNL). The method allows you to visually assess the local capillary network of the nail bed and identify structural changes in capillaries and impaired capillary blood flow. In primary SR, there are no structural changes in the capillaries, but functional disorders are detected in the form of a pronounced decrease in the blood flow velocity in the capillaries or intracapillary stasis (Fig. 1A). Secondary SR is characterized by changes in the size and shape of capillary loops, reduction of the capillary network (Fig. 1B).
In order to differentiate primary and secondary SR, a number of other instrumental studies are also used.
Laser Doppler flowmetry - assessment of skin blood flow; the use of provocative tests reveals increased vasospasm and a decrease in vasodilation potential.
Thermography - an indirect assessment of blood flow by the level of skin temperature; the recovery time of the initial skin temperature after cooling and the gradient to along the finger reflect the severity of vascular damage. Temperature differences between the pad of the fingers and the back of the hand of more than 1°C at 30°C have a positive and negative predictive value (70 and 82%, respectively) for the detection of SR secondary to systemic scleroderma (SSD).
Plethysmography - measurement of blood pressure in the digital artery; a decrease in pressure by 70% or more, after local cooling, indicates the secondary nature of SR (97% sensitivity); SJS is characterized by a decrease in pressure to 0 at 30°C (100% specificity).
Color Doppler ultrasound scanning - visualization and measurement of the diameter of the digital artery, assessment of blood flow velocity; allows to differentiate primary and secondary SR.
For early differential diagnosis of primary and secondary SR, it is recommended to follow the algorithm shown in Figure 2.
Differential diagnosis of Raynaud's syndrome should be made primarily with acrocyanosis, a condition characterized by prolonged cyanosis of the hands or feet, which is aggravated by cold.
Some conditions may be mistaken for Raynaud's syndrome. These include carpal tunnel syndrome, reflex sympathetic dystrophy, and superior aperture syndrome. All these syndromes are associated with mechanical damage to the neurovascular bundle of the upper extremities.
Particular attention should be paid to taking drugs such as α-interferon, antitumor agents (cisplatin, bleomycin, vinblastine, etc.), β-blockers and bromocriptine.
Livedo reticularis is also observed in vasculitis, antiphospholipid syndrome, and occlusive peripheral vascular disease, in which, unlike SR, this symptom is stable.
Freezing limbs, their numbness and tingling are often complained by patients with peripheral vascular disease, accompanied by a decrease in blood flow and ischemia. In SR, in contrast to peripheral vascular disease, these symptoms are observed only during vasospasm and completely disappear after the restoration of the initial blood flow.
Course and forecast
Primary SR has a favorable course and prognosis. At the same time, during long-term follow-up of 307 women with SR (average 12 years), 38% of them showed no changes, 36% showed a decrease in the frequency and severity of Raynaud's attacks, in 16%, on the contrary, an increase in the severity of clinical symptoms, and in 10% - manifestations of SR have passed. In a prospective follow-up of patients with SR for 3 years, DeAngelis R, et al. established the development of connective tissue disease in 10% of patients with an initial diagnosis of probably secondary SR and in no case of primary SR. With a longer follow-up, for 12 years, the development of connective tissue disease was noted already in 30% of patients with probably secondary SR, as well as in 9% of patients with primary SR. The Framingham Study showed that 81 of 639 (12.6%) patients with SR developed signs of another disease after a median of 10.4 years (0.6-27.9 years). The development of systemic scleroderma (in 53 of 81 or 65% of patients) and mixed connective tissue disease (in 8 of 81 or 10% of patients) was most often noted. In secondary SR, the prognosis is determined primarily by the disease with which the association is noted.
Treatment
All patients with both primary and secondary Raynaud's syndrome are advised to exclude cooling, smoking, contact with chemical and other factors that provoke vasospasm in everyday life and at work. Stressful situations, sudden changes in temperature should be avoided, keep the whole body warm, and especially the hands and feet (wear warm clothes, a hat, mittens instead of gloves, thermal underwear, etc.). Identification and elimination of provoking factors (cold, vibration, etc.) is the basis for the treatment of professional RS. Treatment of the underlying disease, aimed at eliminating vascular disorders, reducing the activity of the pathological process, has a positive effect on the manifestations of secondary SR.
In cases of frequent and prolonged episodes of vasospasm in primary SR and in almost all cases of secondary SR, drug therapy is necessary. For the treatment of SR, drugs with a vasodilatory effect or drugs that affect the rheological properties of the blood are used.
Among vasodilators, calcium channel blockers are effective in the treatment of SR, which are considered first-line drugs for SR; the drug of choice is nifedipine, which is prescribed at a dose of 30–60 mg/day in separate courses or for a long time. A third of patients may experience side effects: refractory tachycardia, headache, facial flushing, swelling of the ankles, etc. Preference is given to long-acting nifedipine due to a decrease in the frequency of adverse events. With intolerance to nifedipine, it is possible to prescribe other calcium channel blockers (amlodipine, isradipine and felodipine, related to long-acting drugs), but their therapeutic effect is somewhat lower. Amlodipine is prescribed once a day at a dose of 5 mg, with insufficient effect, the daily dose can be increased to 10 mg. The most common side effect of amlodipine is ankle swelling. Isra-dipin is prescribed at a dose of 2.5 mg 2 times a day. Side effects in the form of headaches and flushing are usually moderate in nature. Felodipine is used at a dose of 10 mg 1 time per day, mainly in the form of dosage forms that provide a gradual release of the drug substance. Calcium channel blockers inhibit platelet activation, which also has a beneficial effect in CP.
The effect of drugs is expressed in a decrease in the frequency of SR attacks and their duration, and its long-term use leads to the reverse development of vascular-trophic disorders. Compared with the secondary in patients with primary SR, the therapeutic effect is usually manifested to a greater extent. With prolonged use of nifedipine in large doses, paresthesia, muscle pain, as well as the development of tolerance and a decrease in the therapeutic effect of the drug are possible.
With the progressive nature of SR, the use of Vasaprostan® (prostaglandin E1) is recommended, which has a pronounced vasodilating effect, inhibits platelet activity and aggregation, reduces thrombosis, has a positive effect on the endothelium, etc.
The drug is administered intravenously at a dose of 20-40 mcg of alprostadil in 100-200 ml of saline for 1-2 hours daily; for a course of 15-20 infusions. The initial effect may appear after 2-3 infusions, but a more persistent effect is noted after the end of the course of therapy and is expressed in a decrease in the frequency, duration and intensity of Raynaud's syndrome attacks, a decrease in chilliness, numbness and ischemic pain, as well as ulcerative necrotic changes in the extremities , up to complete healing of ulcers in 1/3 of patients. The positive effect of Vasa-prostan® usually lasts for 4-6 months; it is recommended to conduct repeated courses of treatment (2 times a year).
With insufficient effect, EULAR experts recommend adding infusion prostanoids (iloprost and intravenous epoprostenol - ilomidin) to the treatment of vascular disorders. The drugs have a pronounced vasodilating effect, are used to treat severe Raynaud's syndrome, mainly secondary, associated with systemic scleroderma, with active digital ulcers, pulmonary hypertension.
Of the other drugs that have a pronounced vasodilatory effect, blockers of type I angiotensin II receptors (losartan) can be noted. The effect of the drug was more pronounced in patients with primary SR, was to reduce the frequency of vasospasm attacks.
In the treatment of SR, a number of vasodilators (transdermal nitroglycerin, hydralazine, papaverine, minoxidil, nicotinic acid derivatives) are used, which can be effective in selected patients, mainly in primary SR. However, the frequent development of side effects (systemic hypotension, headache) limits the use of these drugs. The results of studies on the effectiveness of angiotensin-converting enzyme inhibitors in patients with SR are very controversial. Currently, drugs of this group have not found wide application in clinical practice.
In the complex treatment of primary SR, angioprotectors and Ginkgo Biloba, a herbal preparation with a moderate vasoactive effect (reduces the number of SR attacks with prolonged use), can also be used.
The use of sympatholytic drugs is justified by the fact that adrenergic stimulation plays an important role in vasoconstriction. Prazosin significantly reduces the severity and frequency of vasospasm in patients with primary SR.
Of great importance in the treatment of secondary SR are drugs that improve the rheological properties of blood, reduce viscosity and have an antiaggregation effect: dipyridamole 75 mg or more per day; pentoxifylline at a dose of 800-1200 mg / day orally and intravenously; low molecular weight dextrans (reopoliglyukin, etc.) - intravenous drip of 200-400 ml, 10 infusions per course. It is possible to use anticoagulants, more often in secondary SR, in the presence of signs of thrombosis.
In the treatment of SR, one should take into account the need for long-term multi-year therapy and often the complex use of drugs from different groups.
It is recommended to combine SR drug therapy with the use of other methods of treatment: hyperbaric oxygen therapy, reflexology, psychotherapy, physiotherapy, digital sympathectomy. Sympathectomy can be used in the absence of a rapid effect of drug therapy, as an auxiliary measure in the complex treatment of severe SR. Temporary chemical sympathectomy (lidocaine, etc.) may be preferred as an adjunct to acute vasospasm in critical digital ischemia; active drug therapy continues and may be more successful.
Thus, the doctor has at his disposal a fairly wide arsenal of therapeutic effects on SR and associated vascular-trophic disorders.
In most cases, SR is a disease characterized by a favorable prognosis and a stable course. At the onset of the disease, especially in the presence of risk factors of its secondary nature, all patients with SD are subject to clinical examination and medical examination once a year. Patients should be warned about the need for an additional visit to the doctor if new symptoms appear that indicate the possible development of diseases with which SR is most often associated, primarily systemic connective tissue diseases.

Literature
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13. EC, Medsger TR. Raynaud's phenomenon: a proposal for classification. Clin Exp Rheumatol 1992; 10:485-488] and secondary. In 1/4 of patients, SR occurs in first-line relatives)