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 are contradictory. One of the largest population studies has shown that Raynaud's disease occurs in 21% of women and 16% of men. Among professions where the risk of vibration disease is increased, this percentage is two times higher.

[1],

What causes Raynaud's disease?

Raynaud's disease occurs in different climatic conditions. It is most rare in countries with a hot, constant climate. Incidents of the disease and among the northerners. Raynaud's disease is most common in mid-latitudes, in places with a humid temperate climate.

Hereditary predisposition for Raynaud's disease is small - about 4%.

In its classical form, Raynaud’s symptom complex occurs in the form of attacks consisting of three phases:

1. blanching and cooling of the fingers and toes, accompanied by pain;
2. the addition of cyanosis and increased pain;
3. limb reddening and pain subsiding. Such a symptom complex is usually referred to as Raynaud's phenomenon. All cases of pathognomonic combination of symptoms of a primary disease with physical signs of FR are designated as Raynaud's syndrome (CP).

As shown by clinical observations, 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 (BR).

Among the many attempts to classify various forms of Raynaud's syndromes, the most complete is the one created by L. And P. Langeron, L. Croccel in 1959. The etiopathogenetic classification, which in the modern interpretation is as follows:

1. The local origin of Raynaud's disease (finger arteritis, arteriovenous aneurysm of the vessels of the fingers and toes, professional and other trauma).
2. Regional origin of Raynaud's disease (cervical ribs, anterior scalene muscle syndrome, sharp arm abduction syndrome, damage to the intervertebral disc).
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 systemic disease (arteritis, arterial hypertension, primary pulmonary hypertension).
5. Raynaud's disease with insufficient blood circulation (thrombophlebitis, vein injuries, heart failure, cerebral vascular angiospasm, retinal vasospasm).
6. Damage to the nervous system (constitutional acrodynia, syringomyelia, multiple sclerosis).
7. The combination of Raynaud's disease with digestive disorders (functional and organic diseases of the digestive tract, gastric ulcer, colitis).
8. Raynaud's disease in combination with endocrine disorders (diencephalic-pituitary disorders, adrenal tumors, hyperparathyroidism, Bazedovoy disease, menopausal menopause, as well as menopause as a result of radiation therapy and surgical intervention).
9. Raynaud's disease in violation of blood (congenital splenomegaly).
10. Raynaud's disease with cryoglobulinemia.
11. Raynaud's disease with scleroderma.
12. True Raynaud's Disease.

Later, this classification was supplemented with some selected clinical forms for rheumatic lesions, hormonal dysfunction (hyperthyroidism, postmenopausal period, uterine and ovarian dysplasia, etc.), for 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 hypertension, angina pectoris and other diseases and are capable of causing The symptoms of Raynaud's disease in patients with a corresponding predisposition.

Pathogenesis of Raynaud's disease

The pathophysiological mechanisms underlying the onset of attacks of Raynaud's disease are not fully understood. M. Raynaud considered the cause of the disease described by him to be "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 of loyalty to one of these points of view. The latter assumption has certain grounds in the light of modern ideas about the vascular effects of prostaglandins. It was shown that Raynaud's disease reduces the synthesis of endothelial prostacyclin, the vasodilating effect of which causes no doubt in the treatment of patients with Raynaud's disease of various etiologies.

Currently established is the fact that the rheological properties of the blood in patients suffering from Raynaud's disease, especially during an attack, change. The direct cause of the increase in blood viscosity in these cases is not clear: this may be a result of both changes in plasma fibrinogen concentration and deformation of red blood cells. Similar conditions occur in cryoglobulinemia, in which the link between impaired precipitation of proteins 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, which are manifested in frequent headaches, strokes, and muscular asthenia. The relationship of clinical manifestations of peripheral circulatory disorders with the occurrence and course of emotional disorders is confirmed by a change in digital blood flow in response to emotional stress, the effect of disturbing emotional states on skin temperature in both patients with Raynaud's disease and in healthy people. The state of nonspecific systems of the brain is essential, as was shown by numerous EEG studies in various functional states.

In general, the presence of clinical signs, such as paroxysmality, symmetry, dependence of clinical manifestations on various functional states, the role of the emotional factor in provoking attacks of Raynaud’s disease, some biorhythmic dependence, pharmacodynamic analysis of the disease, allows us to imply the involvement of cerebral mechanisms in the pathogenesis of this suffering.

In Raynaud's disease, a disturbance in the integrative activity of the brain is shown (through the study of ultralow, as well as induced brain activity), manifested by a mismatch between specific and nonspecific processes of somatic afferentation, various levels of information processing, mechanisms of nonspecific activation).

An 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 only in the case of the idiopathic form of the disease, evidence of the insufficiency of the mechanisms of sympathetic segmental regulation of cardiovascular and sudomotor activity. The presence of insufficiency of sympathetic influences in conditions of vasospastic disorders suggests that the existing symptom complex is a consequence of vasospasm, as a phenomenon of post-denervation hypersensitivity. The latter, obviously, has a compensatory-preventive value to ensure a sufficient level of peripheral blood flow and, as a consequence, the preservation of vegetative-trophic functions in Raynaud's disease. The value of this compensatory factor is especially clearly seen when compared with patients with systemic scleroderma, where the percentage of vegetative-trophic disorders is so high.

[2], [3], [4], [5], [6], [7], [8], [9], [10],

Symptoms of Raynaud's Disease

The average age of onset of Raynaud's disease is the second decade of life. The cases of Raynaud's disease in children 10-14 years old are described, about half of which were hereditary. In some cases, Raynaud's disease occurs after emotional stress. The onset of Raynaud's disease after 25 years, especially in individuals who had no previous signs of impaired peripheral circulation, increases the likelihood of having any primary disease. Rarely, usually after severe mental shocks, endocrine rearrangements, the disease can occur in people 50 years and older. Raynaud's disease occurs in 5-10% of those surveyed in the population.

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

Among the factors provoking the occurrence of attacks of Raynaud's disease, the main effect is exposure to cold. In some people with specific features of peripheral blood circulation, even the inconsistent episodic exposure to cold and humidity can cause Raynaud's disease. Emotional experiences are a frequent cause of the onset of attacks of Raynaud's disease. There is evidence that about 1/2 of the patients with Raynaud's disease have a psychogenic nature. 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 autonomic-endocrine system are the background that facilitates the occurrence of Raynaud's disease. Direct inheritance is small - 4.2%.

The earliest symptom of the disease is increased chilliness of the fingers - most often the hands, which then join the blanching of the terminal phalanges and pain in them with elements of paresthesia. These disorders are paroxysmal in nature and at the end of the attack disappear completely. The distribution of peripheral vascular disorders does not have a strict pattern, but more often it is the II-III fingers of the hands and the first 2-3 fingers of the feet. Distal parts of the arms and legs are most involved in the process, much less often other parts of the body - ear lobes, nose tip.

The duration of attacks is different: more often - a few minutes, less often - a few hours.

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

The course of the disease is slowly progressing, 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, changes in climatic conditions.

The described stages of development of the disease are characteristic of secondary Raynaud's disease, where the rate of progression is determined by the clinical picture of primary suffering (most often it is a systemic disease of the connective tissue). The course of Primary Raynaud's disease, as a rule, is inpatient.

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

Arterial hypertension is observed in 9% of cases.

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

Despite the significant number of complaints about the 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 idiopathic form of the disease are extremely rare.

Numerous studies of the idiopathic form of Raynaud's disease showed the complete patency of the great vessels, and therefore the severity and frequency of convulsive crises of arteriocapillaries in the distal parts of the extremities remain difficult to explain. Oscillography only shows an increase in the tone of blood vessels, mainly of the hands and feet.

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

1. in the compensation stage, a marked increase in vascular tone;
2. in the stage of decompensation - most often a significant decrease in the tone of the small arteries and veins. Pulse blood circulation decreases in the fingers of the hands and feet during the period of an ischemic attack with symptoms of venous outflow.

[11], [12], [13]

Diagnosis of Raynaud's Disease

In the study of patients with Raynaud's disease, it should first 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 different intensity. For many people, it includes single-phase blanching of the fingers or toes. This reaction undergoes 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 stimuli.

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 the five main criteria formulated by E.Ellen, W.strongrown in 1932:

1. disease duration of at least 2 years;
2. the absence of diseases secondary to Raynaud's syndrome;
3. strict symmetry of vascular and tropho-paralitic symptoms;
4. lack of gangrenous skin changes of the fingers;
5. episodic occurrence of attacks of ischemia of the fingers under the influence of cooling and emotional experiences.

However, further, with a disease duration of more than 2 years, it is necessary to exclude systemic diseases of the connective tissue, as well as other most common causes of secondary Raynaud's disease. Therefore, special attention should be paid to the identification of symptoms such as thinning of the terminal phalanges, multiple long-term 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 the possible diagnosis of systemic scleroderma. Systemic lupus erythematosus is characterized by a butterfly-shaped erythema on the face, increased sensitivity to sunlight, hair loss, and symptoms of pericarditis. The combination of Raynaud's disease with dryness of the mucous membranes of the eyes and mouth is characteristic of Sjogren's syndrome. In addition, patients should be interviewed in order to identify in the history of data on the use of drugs such as ergotamine and long-term treatment with beta-blockers. In men older than 40 years old with a decrease in peripheral pulses, it is necessary to find out if they smoke, in order to establish a possible connection between Raynaud's disease and obliterating endarteritis. To exclude professional pathology, anamnestic data on the ability to work with vibrating instruments is mandatory.

In almost all cases of the patient's primary treatment to the doctor, it is necessary to carry out a differential diagnosis between its two most common forms - idiopathic and secondary with systemic scleroderma. The most reliable method, along with detailed clinical analysis, is the application of the method of induced skin sympathetic potentials (HVTS), which allows to demarcate these two states almost 100%. In patients with systemic scleroderma, these indicators practically do not differ from normal. While in patients with Raynaud's disease, there is a sharp lengthening of the latent periods and a decrease in the amplitudes of HKSP in the limbs, more roughly represented on the arms.

Vascular-trophic phenomena in the extremities are found in various clinical variants. The phenomenon of acroparesthesia in a milder form (Schulz form) and more severe, common, with symptoms of edema (Notnagel form) is limited to subjective sensitivity disorders (goosebumps, tingling, numbness). The state of persistent acrocyanosis (Cassirer's acroasphyxia, local sympathetic asphyxia) can be aggravated and occur with signs of puffiness, mild hypoesthesia. There are numerous clinical descriptions of Raynaud's disease, depending on the location and prevalence of angiospastic disorders (the phenomenon of "dead finger", "dead hand", "foot of a miner", etc.). Most of these syndromes are united by a number of common features (paroxysmality, the appearance under the influence of cold, emotional stress, a similar nature of the course), which suggests that they have a single pathophysiological mechanism and treat them within a single Raynaud's disease.

Raynaud's disease treatment

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

Most of the conventional methods relate to symptomatic forms of treatment, based on the use of general strengthening, antispasmodic painkillers and normalizing hormonal functions of the remedies.

Special management and treatment tactics 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 the primary Raynaud's disease is caused only by cold, humidity and emotional stress, the exclusion of these factors may lead to the relief of attacks of Raynaud's disease. Analysis of clinical observations of the results of long-term administration of various groups of vasodilators indicates their lack of effectiveness and short-term clinical improvement.

The use of defibrinating therapy as a type of pathogenetic treatment is possible, but its short-term effect should be kept in mind; 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, a significant relief comes from surgical treatment - sympathectomy. Observations on operated patients show that the return of almost all symptoms of the disease is observed after a few weeks. The rate of increase in symptoms coincides with the development of hypersensitivity of denervated structures. From these positions it becomes clear that the use of sympathectomy is not at all justified.

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

Of particular interest from the pathogenetic point of view is the use of large doses of cycloxide inhibitors (indomethacin, ascorbic acid) in order to correct disturbances in the peripheral circulation.

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

To date, there have been some new aspects of the treatment of patients with Raynaud's disease. With the help of biofeedback, patients manage to control and maintain skin temperature at a certain level. Autogenic training and hypnosis provide a special effect in patients with Raynaud's idiopathic disease.

[14], [15]