TAKAYASU SYNDROME

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TAKAYASU SYNDROME (M of Takayasu, Japanese doctor, sort. in 1872; synonym: arteritis of young women, disease of lack of pulse, Takayasu's disease, nonspecific aorto-arteritis, return coarctation of an aorta, primary arteritis of an aortic arch, aortic arch syndrome) — nonspecific obliterating aortoarteriit or the brakhiotsefalny arteritis which is shown pressure decrease, weakening of pulse and reduction of a blood-groove in stenosed arteries of an upper half of a trunk. Patol. process is localized not only in an aortic arch and vessels departing from it, it has system character and can cover the descending part of an aorta throughout and the large arteries departing from it.

To Takayasu (1908) message on observation of changes of arteries of a retina of an eye in combination with lack of pulse on a beam artery at the young woman this syndrome was repeatedly described since the middle of 19 century, including in Russia (S. S. Yakovleva, 1884). It was accepted to call a syndrome by the name of Takayasu in Japan where the number of its descriptions (but not prevalence) to the middle of 20 century was prevailing. It is tended to designate as an aortic arch syndrome only cases at young women, and other options of this pathology — to call «aortic arch syndrome» or «a disease of lack of pulse».

An etiology and a pathogeny

If the obliteration of arteries described as T. the page, has a specific etiology (tuberculosis, syphilis), it is considered within the main disease. For a nonspecific aortoarteriit there was standard a hypothesis of autoimmune genesis, in advantage a cut testify quite often observable at patients hron. inflammatory processes, serum disease (see), small tortoiseshell (see), polyarthritis (see), development of diseases after pregnancy, long insolation and other influences changing the immune status. The role of autoimmune processes is confirmed by frequent detection in blood serum of sick anti-aortal antibodies in high credits, and also results immunol. researches using a method of fluorescent antibodies. Believe that fabrics of an arterial wall gain antigenic properties in connection with damage them inf. process. Role of features inf. the agent it is not studied, but it is possible to assume that his ability to damage nuclear structures of cells matters or to form an antigenic complex with elements of an endothelium of arteries.

Pathological anatomy

Morfol. the essence of a disease consists in development of the obliterating arteritis affecting all layers of a wall of the vessels departing from an aorta, and which is preferential localized in mouths. In this regard the greatest distribution was gained by the name «nonspecific aorto-arteritis», most correct reflecting kliniko-morphological essence of process (see. Aortitis , Arteritis ).

Histologically signs of an inflammation (see) decide on dominance proliferative inflame - telnykh changes in all layers of walls of an aorta and its branches; lymphocytic infiltration, existence of a large number of plasmocytes, signs of fibrinoid swelling, fibrinoid necroses in an adventitia of vessels are characteristic. Important feature of T. page — simultaneous existence in a vascular wall of various phases and forms of inflammatory process: the acute stage of an inflammation in one site of a vascular wall is quite often combined with sclerous changes in another. The described changes extend to an internal cover (intima) in the last turn. Defeat of an internal cover of arteries is complicated by widespread pristenochny, quite often obliterating thrombosis (see).

Clinical picture

Polymorphism of anatomic options of localization and distribution patol. process the wedge, manifestations of T causes variability. page. On preferential localization it is possible to allocate four main options of defeat, not all from to-rykh correspond to classical manifestation of T. page: aortokarotidny, aortal, system option and the isolated stenosis of one artery.

Aortokarotidny option T. the page is the basic; at it the aortic arch is surprised and the arteries departing from it are stenosed. It is shown by asymmetry of the ABP, absence or weakening of pulse on one or both hands that can be followed by weakness in a hand. At damage of arteries of the head dizzinesses, faints, decrease in sight are observed and changes of an eyeground are defined. One of symptoms of aortokarotidny option of an aortic arch syndrome is the coronary insufficiency (see) connected with defeat of coronary arteries. At the same time in 2/3 cases patients do not show typical complaints to pain in heart.

The aortal option (defeat of a belly part of an aorta and the main arteries departing from it — main renal, mezenterialny, ileal, etc.) is clinically characterized as the general signs of damage of various arteries (systolic noise in the field of a projection of the struck vessel, existence of network of collaterals), and the features inherent to defeat of each of them. Change of the main renal arteries is one of the reasons of arterial hypertension (see arterial hypertension). Damage of mezenterialny arteries is shown by an abdominal pain, weight loss and an unstable chair. At an obliteration of ileal arteries onychalgias and the alternating lameness are noted (see. Obliterating defeats of vessels of extremities).

System option aortoarterii-that is characterized by symptoms of defeat of chest and belly parts of an aorta, and also the main arteries, including the main renal departing from them.

The isolated stenosis, or occlusion only of one of large arteries — a pulmonary trunk, subclavial, sleepy, renal, etc., is distinguished on characteristic displays of ischemia of the corresponding fabrics, signs of a vascular stenosis — to noise (see. Vascular noise), changes of cardiodynamics etc.

Complications depend on localization and the nature of defeat of vessels. The most serious of them are arterial hypertension, heart attacks of heart and brain (see the Stroke, the Myocardial infarction) in connection with coronary insufficiency and ischemia of a brain, heart failure (see), to-rye can be also the main reasons for death at T. page.

Diagnosis is established, as a rule, at already created obliteration of vessels. In the main stage of a disease of its manifestation are characterized by nonspecific inflammatory reactions, to-rye connect with a vasculitis (see) only in the presence of strong indications of vascular defeat. At an obliteration and formation of stenoses of arteries the diagnosis of T. the page is established on the basis of the available vascular trunks given to a palpation, auscultation of vascular noise, measurement of the ABP standing and hands (detection of asymmetry), and also given to an aortografiya (see), coronary angiography (see). On angiograms deformation and an aneurysmal aortectasia with a stenozirovaniye or occlusion of mouths of the main branches, powerful collateral vascular network comes to light. Also laboratory signs of an inflammation (a leukocytosis, acceleration of ROE, increase in blood of level of gamma-globulins) and immu-nol are considered. shifts (change in composition of immunoglobulins, detection of the circulating immunocomplexes).

Differential diagnosis carry out first of all with atherosclerotic defeat of vessels (see. And those-roskleroz), a cut unlike T. the page meets more often at men 40 years are more senior. Damage of arteries of small and average caliber, generally lower extremities is characteristic of an obliterating endarteritis (see the Endarteritis obliterating). Stenozirovaniye of renal arteries can be a consequence of a fibrous dysplasia, however at the same time the aorta is never surprised.

Treatment

Due to the autoimmune genesis of a disease in each case of T. page an attempt of treatment shall be made by glucocorticoids, a cut carry out in a hospital.

At the same time the diagnosis of a disease shall be verified angiographically or during operational intervention. Glyukokortikoidg begin to enter with small doses, to-rye gradually increase, bringing to the dose equivalent 40 mg predni-zo l it in days, then slowly reducing by the end of a course of treatment. On a course of treatment lasting 35 — 40 days 1,3 — 1,5 g of glucocorticoids in terms of Prednisolonum are necessary. Contraindications for use of glucocorticoids are high ABP, the malignant course of renovascular hypertensia and the related complication and also a peptic ulcer of a stomach.

Besides, apply heparin on 10 000 PIECES 2 — 3 times a day intramusculary within 10 — 20 days under control of a blood clotting time; the means improving microcirculation (e.g., trental) and possessing anti-aggregation action. In cases, proceeding with the high ABP, and also at weak expressiveness of basic process it is recommended it is long (4 — 5 months) to apply indometacin in a dose of 75 — 100 mg a day in out-patient conditions. Systematically carried out drug treatment allows to achieve approximately in 70% of cases improvement of a state and stabilization of process.

At rather isolated process and disturbance of blood supply of bodies, important for life (the expressed vertebrobazilyarny insufficiency), and also at renovascular hypertensia operative measures are shown various look: prosthetics, shunting, an endarteriektomiya and so forth (see Blood vessels, operations; Subclavial artery, operations; Carotid arteries; Shunting of blood vessels). The expressed activity of process is a relative contraindication for operation. An operative measure does not exclude need of carrying out a pathogenetic drug and symptomatic treatment.

Forecast at T. the page striking generally people of young age can be satisfactory on condition of early diagnosis of a disease and systematic treatment, a cut quite often allows to improve a condition of patients and to stabilize at least patol. process. The correct organization of drug treatment, timely surgery if necessary, and also dispensary observation of the cardiologist improve the vital forecast: life expectancy of patients (from the moment of observation) can reach 20 — 25 years.



Bibliography: Abugova S. P., etc. Coronary insufficiency at tsanarterii-those, Cardiology, t. 18, No. 5, page 61, 1978; Arabidze G. G., etc. Clinical aspects of a disease of Takayasu, Rubbed. arkh., t. 52, No. 5, page 124, 1980; Pokrovsky A. V. Diseases of an aorta and its branches, M., 1979.


G. G. Arabidze, S. P. Abugov.

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