Raynaud's disease

Raynaud's disease occupies a leading place among the group of vegetative-vascular diseases of the distal extremities.

Data on the prevalence of Raynaud's disease is conflicting. One of the largest population studies found that Raynaud's disease affects 21% of women and 16% of men. Among professions with a higher risk of developing vibration syndrome, this percentage is twice as high.

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What causes Raynaud's disease?

Raynaud's disease occurs in a variety of climates. It is rarest in countries with hot, constant climates. There are also rare reports of the disease among northerners. Raynaud's disease is most common in mid-latitudes, in places with a humid, temperate climate.

The hereditary predisposition to Raynaud's disease is small - about 4%.

In its classical form, Raynaud's syndrome occurs in attacks consisting of three phases:

1. paleness and coldness of the fingers and toes, accompanied by pain;
2. the addition of cyanosis and increased pain;
3. redness of the extremities and abatement of pain. Such a symptom complex is usually referred to as Raynaud's phenomenon. All cases of pathognomonic combination of symptoms of the primary disease with physical signs of RP are referred to as Raynaud's syndrome (RS).

As clinical observations have shown, the symptom complex described by M. Raynaud is not always an independent disease (idiopathic): it can also occur in a number of diseases that differ in etiology, pathogenesis and clinical manifestations. The disease described by M. Raynaud began to be regarded as an idiopathic form, i.e. Raynaud's disease (RD).

Among the numerous attempts to classify the various forms of Raynaud's syndromes, the most complete is the etiopathogenetic classification created by L. and P. Langeron, L. Croccel in 1959, which in its modern interpretation looks like this:

1. Local origin of Raynaud's disease (digital arteritis, arteriovenous aneurysms of the vessels of the fingers and toes, professional and other trauma).
2. Regional origin of Raynaud's disease (cervical ribs, anterior scalene syndrome, arm abduction syndrome, intervertebral disc disease).
3. Segmental origin of Raynaud's disease (arterial segmental obliteration, which can cause vasomotor disorders in the distal extremities).
4. Raynaud's disease in combination with a systemic disease (arteritis, arterial hypertension, primary pulmonary hypertension).
5. Raynaud's disease due to insufficient blood circulation (thrombophlebitis, venous trauma, heart failure, cerebral vasospasm, retinal vasospasm).
6. Lesions of the nervous system (constitutional acrodynia, syringomyelia, multiple sclerosis).
7. Combination of Raynaud's disease with digestive disorders (functional and organic diseases of the digestive tract, stomach ulcer, colitis).
8. Raynaud's disease in combination with endocrine disorders (diencephalic-pituitary disorders, adrenal tumors, hyperparathyroidism, Graves' disease, climacteric menopause, as well as menopause as a result of radiation therapy and surgery).
9. Raynaud's disease due to hematopoiesis disorder (congenital splenomegaly).
10. Raynaud's disease in cryoglobulinemia.
11. Raynaud's disease in scleroderma.
12. True Raynaud's disease.

Later, this classification was supplemented by some isolated clinical forms in rheumatic lesions, hormonal dysfunction (hyperthyroidism, post-menopausal period, dysplasia of the uterus and ovaries, etc.), in some forms of occupational pathology (vibration disease), complications after taking vasoconstrictor drugs of peripheral action, such as ergotamine, beta-blockers, widely used for the treatment of arterial hypertension, angina pectoris and other diseases and capable of causing attacks of the symptom of Raynaud's disease in individuals with the corresponding predisposition.

Pathogenesis of Raynaud's disease

The pathophysiological mechanisms underlying the occurrence of attacks of Raynaud's disease have not been fully elucidated. M. Raynaud believed that the cause of the disease he described was "hyperreactivity of the sympathetic nervous system." It is also assumed that this is the result of a local defect (local-fault) of the peripheral vessels of the fingers. There is no direct evidence to support either of these points of view. The latter assumption has certain grounds in light of modern concepts of the vascular effects of prostaglandins. It has been shown that in Raynaud's disease there is a decrease in the synthesis of endothelial prostacyclin, the vasodilatory effect of which is beyond doubt in the treatment of patients with Raynaud's disease of various etiologies.

At present, it is an established fact that the rheological properties of blood change in patients suffering from Raynaud's disease, especially during an attack. The immediate cause of the increase in blood viscosity in these cases is not clear: it may be a consequence of both a change in the concentration of plasma fibrinogen and the deformation of red blood cells. Similar conditions occur in cryoglobulinemia, in which the connection between the violation of protein precipitation in the cold, an increase in blood viscosity and the clinical manifestations of acrocyanosis is undeniable.

At the same time, there is an assumption about the presence of angiospasms of the cerebral, coronary and muscular vessels in Raynaud's disease, the manifestation of which are frequent headaches, attacks of angina pectoris, and muscular asthenia. The connection between the clinical manifestations of peripheral circulatory disorders and the occurrence and course of emotional disorders is confirmed by changes in digital blood flow in response to emotional stress, the influence of anxious emotional states on skin temperature in both patients with Raynaud's disease and healthy people. The state of non-specific brain systems is of significant importance, which has been shown by numerous EEG studies in various functional states.

In general, the very presence of clinical signs, such as paroxysmal nature, symmetry, dependence of clinical manifestations on various functional states, the role of the emotional factor in provoking attacks of Raynaud's disease, some biorhythmological dependence, pharmacodynamic analysis of the disease, allows us to assume with sufficient reason the participation of cerebral mechanisms in the pathogenesis of this disease.

In Raynaud's disease, a disturbance of the integrative activity of the brain is shown (using the study of infraslow and evoked brain activity), manifested by a discrepancy between specific and non-specific processes of somatic afferentation, various levels of information processing processes, and mechanisms of non-specific activation).

Analysis of the state of the autonomic nervous system in Raynaud's disease using special methods that allow selective analysis of its segmental mechanisms revealed facts indicating insufficiency of the mechanisms of sympathetic segmental regulation of cardiovascular and sudomotor activity only in the idiopathic form of the disease. The presence of insufficiency of sympathetic influences in conditions of vasospastic disorders allows us to assume that the existing symptom complex is a consequence of vasospasm, as a phenomenon of post-denervation hypersensitivity. The latter obviously has a compensatory and protective significance for ensuring a sufficient level of peripheral blood flow and, as a consequence, the preservation of autonomic-trophic functions in Raynaud's disease. The significance of this compensatory factor is especially clearly seen when compared with patients with systemic scleroderma, where the percentage of autonomic-trophic disorders is so high.

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Symptoms of Raynaud's disease

The average age of onset of Raynaud's disease is the second decade of life. Cases of Raynaud's disease have been described in children aged 10-14, approximately half of whom had a hereditary predisposition. In some cases, Raynaud's disease occurs following emotional stress. The onset of Raynaud's disease after 25 years, especially in individuals who have not previously had signs of peripheral circulatory disorders, increases the likelihood of the presence of some primary disease. Rarely, usually after severe mental shocks, endocrine changes, the disease can occur in people aged 50 and older. Raynaud's disease occurs in 5-10% of those examined in the population.

Among patients suffering from Raynaud's disease, women significantly predominate (the ratio of women to men is 5:1).

Among the factors that provoke attacks of Raynaud's disease, the main one is exposure to cold. In some people with individual features of peripheral circulation, even short-term episodic exposure to cold and humidity can cause Raynaud's disease. Emotional experiences are a common cause of attacks of Raynaud's disease. There is evidence that in about 1/2 of patients, Raynaud's disease is psychogenic. Sometimes this disease occurs as a result of exposure to a whole complex of factors (cold exposure, chronic emotional stress, endocrine-metabolic disorders). Constitutional-hereditary and acquired features of the vegetative-endocrine system are the background that contributes to an easier occurrence of Raynaud's disease. Direct hereditary determinacy is small - 4.2%.

The earliest symptom of the disease is increased coldness of the fingers - most often the hands, which is then accompanied by pallor of the terminal phalanges and pain in them with elements of paresthesia. These disorders are paroxysmal in nature and disappear completely at the end of the attack. The distribution of peripheral vascular disorders does not have a strict pattern, but most often these are the II-III fingers of the hands and the first 2-3 toes. The distal parts of the arms and legs are involved in the process more than others, much less often other parts of the body - the earlobes, the tip of the nose.

The duration of attacks varies: most often - several minutes, less often - several hours.

The listed symptoms are characteristic of the so-called stage I of Raynaud's disease. In the next stage, complaints of asphyxia attacks appear, after which trophic disorders in the tissues may develop: swelling, increased vulnerability of the skin of the fingers. Characteristic features of trophic disorders in Raynaud's disease are their locality, remittent course, and regular development from the terminal phalanges. The last, trophoparalytic stage is characterized by an aggravation of the listed symptoms and the predominance of dystrophic processes on the fingers, face, and toes.

The course of the disease is slowly progressive, however, regardless of the stage of the disease, cases of reverse development of the process are possible - at the onset of menopause, pregnancy, after childbirth, or changes in climatic conditions.

The described stages of disease development are characteristic of secondary Raynaud's disease, where the rate of progression is determined by the clinical picture of the primary disease (usually systemic diseases of connective tissue). The course of primary Raynaud's disease is usually stationary.

The frequency of nervous system disorders in patients with Raynaud's disease is high, reaching 60% in the idiopathic form. As a rule, a significant number of neurotic complaints are detected: headache, feeling of heaviness in the head, pain in the back, limbs, frequent sleep disorders. Along with psychogenic headache, paroxysmal vascular headache is characteristic. Migraine attacks occur in 14-24% of patients.

In 9% of cases, arterial hypertension is observed.

Paroxysmal pain in the heart region is functional in nature and is not accompanied by changes in the ECG (cardialgia).

Despite the significant number of complaints about increased sensitivity of the forearms, fingers and toes to cold, the frequency of sensations of itching, burning and other paresthesias, objective sensitivity disorders in patients with the idiopathic form of the disease are extremely rare.

Numerous studies of the idiopathic form of Raynaud's disease have shown complete patency of the main vessels, which makes the severity and frequency of convulsive crises of arteriolocapillaries in the distal parts of the extremities difficult to explain. Oscillography only demonstrates an increase in vascular tone, mainly in the hands and feet.

Longitudinal segmental rheography of the limbs reveals two types of changes:

1. in the compensation stage - a noticeable increase in vascular tone;
2. in the decompensation stage - most often a significant decrease in the tone of small arteries and veins. Pulse blood filling decreases in the fingers and toes during an ischemic attack with signs of difficulty in venous outflow.

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Diagnosis of Raynaud's disease

When examining patients with Raynaud's disease, it is first necessary to establish whether the phenomenon is a constitutional feature of the peripheral circulation, i.e. a normal physiological reaction to cold of varying intensity. In many people, it includes a single-phase blanching of the fingers or toes. This reaction is subject to reverse development upon warming and almost never progresses to cyanosis. Meanwhile, in patients with true Raynaud's disease, the reverse development of vasospasm is difficult and often lasts longer than the application of the acting stimulus.

The greatest difficulty is the differential diagnosis between the idiopathic form of the disease and secondary Raynaud's syndrome.

Diagnosis of the idiopathic form of the disease is based on five main criteria formulated by E. Ellen, W. Strongrown in 1932:

1. duration of the disease is not less than 2 years;
2. absence of diseases that secondarily cause Raynaud's syndrome;
3. strict symmetry of vascular and trophoparalytic symptoms;
4. absence of gangrenous changes in the skin of the fingers;
5. episodic occurrence of attacks of finger ischemia under the influence of cold and emotional experiences.

However, if the disease lasts for more than 2 years, it is necessary to exclude systemic connective tissue diseases, as well as other most common causes of secondary Raynaud's disease. Therefore, special attention should be paid to identifying symptoms such as thinning of the terminal phalanges, multiple long-term non-healing wounds on them, difficulty opening the mouth and swallowing. In the presence of such a symptom complex, one should first of all think about a possible diagnosis of systemic scleroderma. Systemic lupus erythematosus is characterized by butterfly-shaped erythema on the face, increased sensitivity to sunlight, hair loss, and symptoms of pericarditis. The combination of Raynaud's disease with dry mucous membranes of the eyes and mouth is characteristic of Sjögren's syndrome. In addition, patients should be questioned to identify in the anamnesis data on the use of drugs such as ergotamine and long-term treatment with beta-blockers. In men over 40 years of age with a decrease in peripheral pulse, it is necessary to find out whether they smoke, to establish a possible connection between Raynaud's disease and obliterating endarteritis. To exclude professional pathology, anamnestic data on the possibility of working with vibrating instruments are required.

In almost all cases of the patient's initial visit to the doctor, it is necessary to conduct a differential diagnosis between its two most common forms - idiopathic and secondary in systemic scleroderma. The most reliable method, along with a detailed clinical analysis, is the use of the method of evoked skin sympathetic potentials (ESP), which allows almost 100% differentiation of these two conditions. In patients with systemic scleroderma, these indicators are practically no different from normal. While in patients with Raynaud's disease, a sharp extension of latent periods and a decrease in the amplitudes of ESP in the extremities is noted, more roughly represented in the hands.

Vascular-trophic phenomena in the extremities occur in various clinical variants. The phenomenon of acroparesthesia in a milder form (Schulze form) and a more severe, widespread form, with edema (Nothnagel form) is limited to subjective sensitivity disorders (crawling, tingling, numbness). States of constant acrocyanosis (Cassirer acroasphyxia, local sympathetic asphyxia) can worsen and occur with edema, mild hypesthesia. There are numerous clinical descriptions of Raynaud's disease depending on the localization and prevalence of vasospasmodic disorders (the phenomenon of "dead finger", "dead hand", "miner's foot", etc.). Most of these syndromes are united by a number of common features (paroxysmal, occurrence under the influence of cold, emotional stress, similar course), which allows us to assume that they have a single pathophysiological mechanism and consider them within the framework of a single Raynaud's disease.

Treatment of Raynaud's disease

Treatment of patients with Raynaud's disease presents certain difficulties associated with the need to establish the specific cause that caused the syndrome. In cases where a primary disease is identified, the tactics of patient management should include treatment of the underlying disease and observation by the appropriate specialist (rheumatologist, vascular surgeon, endocrinologist, dermatologist, cardiologist, etc.).

Most of the generally accepted methods refer to symptomatic forms of treatment based on the use of general tonics, antispasmodic painkillers and hormonal function normalizing agents.

Special tactics of management and treatment should be followed in patients with occupational and domestic hazards, while first of all eliminating the factor causing these disorders (vibration, cold, etc.).

In cases of the idiopathic form of the disease, when primary Raynaud's disease is caused only by cold, humidity and emotional stress, the exclusion of these factors can lead to relief of Raynaud's attacks. Analysis of clinical observations of the results of long-term use of various groups of vasodilators indicates their insufficient effectiveness and short-term clinical improvement.

It is possible to use defibrinating therapy as a type of pathogenetic treatment, but its short-term effect should be taken into account; plasmapheresis, used in the most severe cases, has a similar effect.

In some forms of the disease, accompanied by the formation of irreversible trophic disorders and severe pain syndrome, significant relief is provided by surgical treatment - sympathectomy. Observations of operated patients show that the return of almost all symptoms of the disease is observed after several weeks. The rate of symptomatic growth coincides with the development of hypersensitivity of denervated structures. From this position, it becomes clear that the use of sympathectomy is not justified at all.

Recently, the range of applied means of peripheral vasodilation has expanded. The use of calcium blockers (nifedipine) in primary and secondary Raynaud's disease is successful due to their effect on microcirculation. Long-term use of calcium blockers is accompanied by a sufficient clinical effect.

Of particular interest from a pathogenetic point of view is the use of high doses of cyclooxygen inhibitors (indomethacin, ascorbic acid) for the purpose of correcting peripheral circulation disorders.

Given the frequency and severity of psychovegetative disorders in Raynaud's disease, psychotropic therapy occupies a special place in the treatment of these patients. Among the drugs in this group are tranquilizers with anxiolytic action (tazepam), tricyclic antidepressants (amitriptyline) and selective serotonin antidepressants (methaneserine).

By now, some new aspects of therapy for patients with Raynaud's disease have been developed. With the help of biofeedback, patients can control and maintain skin temperature at a certain level. Autogenic training and hypnosis have a special effect on patients with idiopathic Raynaud's disease.

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