Takayasu's disease

Description:

Takayasu's disease (nonspecific aortoarteriit) - a granulematozny inflammation of an aorta and its main branches with development of their occlusion and lack of pulse on one or both hands.
Women aged up to 40-50 years are ill preferential. The disease is described by Tokayasu in 1908. Incidence makes 1.2-3.6 cases on 1.000.000 the population.
Distinguish 4 anatomic types of a disease:

    * The I type - the isolated defeat of an aortic arch and the arteries departing from it, usually at the same time is noted a combination of pathology of the left subclavial and left general carotid artery;
    * The II type - the isolated defeat of chest or belly department of an aorta and its branches;
    * The III type - the combined defeat of an aortic arch and its branches with changes in chest or belly departments of an aorta;
    * The IV type - is involved a pulmonary artery, its branches, at the same time the combination to I, II or the III option is possible.

At Takayasu's disease granulematozny inflammatory process begins in an adventitia and periblasts of a mussel, at microscopic examination of granulomas accumulations of lymphocytes, plasmatic, reticular macrophages are found. Further fibrosis of granulomas, a sklerozirovaniye and anguishes of a mussel, proliferation of an endothelium is observed. The gleam of a vessel is narrowed, conditions for development of blood clot are created.
Immukogenetichesky markers yazlyatsya antigens HLA A10, B5, Bw52, DR2. T lymphocytes take part in development of pathology of arteries at this disease.

Symptoms of the Disease of Takayasu:

  1. The general manifestations - weakness, weight loss, fervescence, drowsiness (in connection with brain ischemia), arthralgias, mialgiya.
  2. Damage of arteries, krovosnabzhayushchy upper extremities - develops a syndrome of ischemia of upper extremities (weakness; hand pains; the feeling of numbness increasing at an exercise stress - a syndrome of "the alternating lameness" of upper extremities); lack of pulse on beam, humeral, subclavial arteries of one or both hands, cold brushes. Sometimes there are pains in the field of the left half of a thorax, the left shoulder, a neck. Are characteristic the expressed difference of arterial pressure on sore and healthy hands, and also more high arterial pressure standing, than on hands is considerable. The affected arteries at a palpation are painful (especially sleepy), over them specific noise is listened.
  3. Defeat of a nervous system is caused by inflammatory process in ekstrakranialny arteries, preferential left general carotid artery, its internal branch, right the general sleepy, and also vertebral arteries. Patients are disturbed by headaches, dizzinesses, a memory impairment, attention and working capacity, a poshatyvaniye when walking, often there are unconscious states. There is also objective focal symptomatology caused by ischemia of the relevant department of TsNS. Detection of a pressure murmur at auscultation of carotid arteries is possible.
  4. Defeat of an organ of sight is shown by decrease in visual acuity, doubling in eyes, the sudden blindness on one eye in connection with occlusion of the central artery of a retina and development of an atrophy of an optic nerve is possible.
  5. Damage of heart is caused by pathological changes of the ascending department of an aorta (consolidation, expansion). At patients aortic incompetence often forms, symptoms of stenocardia in connection with disturbance of blood circulation in coronary arteries appear, development of a myocardial infarction is possible. 50% of patients have a myocarditis leading to development of a chronic circulatory unefficiency.
  6. Arterial hypertension is available for 50% of patients that is caused by ischemia of kidneys, TsNS.
  7. Defeat of a ventral aorta leads to decrease in blood supply of the lower extremities, onychalgias when walking, to emergence of systolic noise on an aorta.
  8. Damage of renal arteries is shown by a proteinuria, is more rare - a hamaturia, development of thrombosis is possible.
  9. Damage of a pulmonary artery is shown by thorax pains, an asthma, ECG signs of pulmonary hypertensia.

Laboratory and tool data:

  1. General blood test: moderate anemia, leukocytosis, increase in SOE.
  2. Biochemical analysis of blood: reduction of level of albumine, at - globulins, seromucoid, a gaptoglobin, cholesterol, lipoprotein fractions.
  3. Definition of HLA antigens. AI of blood: increase in level of immunoglobulins is possible, the Russian Federation in rare instances is found.
  4. Ultrasonic scanning of vessels, an angiography reveal decrease in a blood-groove in the corresponding arteries.
  5. The rheoencephalography reveals decrease in blood supply of a brain, an electroencephalography - decrease in functional capacity of a brain.

Takayasu etiologies:

The etiology of a disease of Takayasu is not represented clear. Now it is considered to be what a disease is the cornerstone inflammatory про­цесс of all layers of a wall of an aorta or its branches (a panaortitis, a panarteritis). In a pathogeny of a zaboleyovaniye autoimmune processes, in particular formation of autoantibodies to aortal antigens with development of vospaliyotelny process, the subsequent sclerosis and an obliteration of a gleam of vessels are important.

Treatment of the Disease of Takayasu:

Treatment of an aortic arch syndrome assumes use of corticosteroids with cytostatics or without them. Treatment depends on activity of a disease. For assessment of activity of a disease the takayas can use the criteria offered Kerr and сотр. Process of which emergence or deterioration in two or bigger numbers of the following symptoms is characteristic is considered active:

    * System manifestations: fevers, arthralgias
    * Increase in SOE
    * Symptoms of ischemia, a vascular wall inflammation - the moving lameness, easing or an izcheznoveniye of pulse, noise over an artery, a karotidiniya, asymmetry of arterial pressure on the right and left extremities
    * Typical angiographic signs.

In an active phase of a disease of a basis of therapy corticosteroids are. Corticosteroids are accepted orally in a dose 1 mg/kg (Prednisolonum), their dose gradually decreases in process of relief of symptoms. Prolonged use of these drugs in a small dose can be necessary. At the same time it is necessary to remember prevention of osteoporosis. At inefficiency goromnalny the terapii of a small number of the patient should add to treatment of a cytostatics. Cytostatics are also applied to a dose decline of glucocorticoids at the chronic course of a disease. Cytostatics at their efficiency are accepted within a year after achievement of remission, then their dose gradually decreases up to cancellation.

    * A methotrexate - 7,5-25 mg/kg a week
    * Azathioprinum - 1-2 mg/kg/days
    * Cyclophosphamide - 2 mg/kg/days (it primeknyatsya at patients with natbyuoly a heavy current and in refractory cases).

According to the small not controlled researches, good addition to corticosteroids at inefficiency of traditional therapy can be considered drugs antagonists of a factor of a necrosis of tumors (ANTI-FNO, or anti-TNF): этанерцепт (25 mg twice a week) and инфликсимаб (3 mg/kg at the beginning of therapy, in 2 weeks, 6 weeks, and then every 8 weeks). Larger randomized research of these drugs at Takayasu's disease is planned.
It is necessary to emphasize importance of correction of classical risk factors cordial сосудитсых diseases: dislipidemiya, hypertensia, addictions. At a vasculitis reception of aspirin in low doses is also recommended.
Surgical treatment of critical stenoses of arteries: angioplasty or surgical revascularization during remission. Indications to surgical treatment:

    * a renal artery stenosis with symptomatic arterial hypertension
    * a stenosis of a coronary artery with myocardium ischemia
    * the moving lameness limiting daily activity
    * brain ischemia
    * heavy coarctation of an aorta, insufficiency of the aortal valve, an aneurysm of chest or belly department of an aorta more than 5 cm in the diameter

Transdermal coronary angioplasty at considerable number of patients in 1-2 years is followed by a restenosis. Aortocoronary shunting is characterized by the best long-term results.