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Arteritis of Takayasi, Takayasu's illness, among often found names of this disease there is "aortic arch syndrome", "nonspecific aortoarteriit" and "an illness of lack of pulse".
The disease represents inflammatory process of an aorta and its branches, but only partial defeat — for example, only an aortic arch and its branches or the descending aorta with the large vessels departing from it is possible. Aortic aneurysms, sometimes multiple can be observed. Perhaps also defeat (in rare instances — isolated) a pulmonary artery or its branches.
The etiology of an illness of Takayasu is not established. Believe that inflammatory damage of an aorta and large vessels happens with the participation of autoimmune mechanisms, about same positive influence on acute displays of a disease of corticosteroids and tsitostatik testifies. In blood serum antibodies in relation to an aorta wall, an endothelium and cardiolipin are found. In some populations of patients communication of a disease with a carriage of certain antigens of the HLA system is noted: DR2 and MB1 (in Japan), DR4 and MVZ (in North America). Pathomorphologically the panaortitis with defeat of all layers of a vascular wall and mainly — an average cover where there is a picture of a productive inflammation is observed; cellular accumulations are located mainly in an outside third of a mussel.
Smooth muscle cells are exposed to destruction, the death of elastic fabric is noted, the periblast is quite often thickened and soldered to surrounding fabrics; the expressed intima hyperplasia is observed. In a chronic stage the sclerosis of a vascular wall with a hyalinosis prevails, it is frequent — aorta calcification. There is a point of view that existence of calcification of an aorta demands an exception of an aortoarteriit from young people.
Clinical displays of an illness of Takayasu
Takayasu's illness is observed at persons of young age, and also children and teenagers can be surprised. Among patients female persons prevail.
The first manifestations are the indisposition, perspiration, arthralgias, mialgiya, temperature increase, weight loss is observed. The inflammatory nature of a disease is confirmed by subfebrile condition, existence at patients of a leukocytosis, the increased content of S-reactive protein, the raised SOE, a hypergammaglobulinemia. Polyserosites (pleurisy, a pericardis, polyarthritis), myocarditis, a glomerulonephritis are possible.
The clinical picture actually of an aortitis is defined by preferential localization of pathological process.
At suspicion on damage of an aorta (any etiology) it is necessary to carry out a palpation of all available vascular areas — arteries of extremities, sleepy, temporal, and auscultation of carotid, subclavial, vertebral, renal, ileal, femoral arteries, and also pas aortas all its extent.
At an aortostenosis or an artery more than for 60% over it there is a systolic noise; the same noise can be observed at vessel aneurism.
At the most frequent option of an illness of Takayasu — defeat of an aortic arch and its branches (aortic arch syndrome) at patients headaches, dizzinesses, vision disorders (sometimes resistant and heavy) are observed, up to total loss of sight; there can be a focal neurologic symptomatology. Often there is a symmetric damage of arteries, for example, of two subclavial and/or two sleepy. Syncopal states which are caused as brain ischemia owing to occlusion of pretserebralny arteries, and vasodepressor reflexes as a result of defeat of a sinocarotid zone are very characteristic. At most of patients absence or weakening of pulse on one of hands is noted (most often at the left), sometimes there is no pulsation of a carotid, superficial temporal artery. Asymmetry of arterial pressure on hands is observed; in the absence of pulse on a hand arterial pressure on it can not be defined. Systolic noise over carotid, subclavial arteries, over a ventral aorta is listened. At damage of a subclavial artery "the alternating lameness" of upper extremities, Reynaud's syndrome can be observed.
At damage of the descending aorta in chest department quite often there is its stenozirovaniye — usually below the left subclavial artery and up to diaphragm level. There is a koarktatsionny syndrome — arterial hypertension in an upper half of a trunk. Arterial pressure on hands increases, on the lower extremities — is lowered. Patients are disturbed by headaches, dizzinesses and other displays of arterial hypertension, at the same time they note a chill and fast fatigue of the lower extremities, and the pulsation on arteries of feet is weakened. At simultaneous damage of the descending aorta and branches of an aortic arch sharply expressed asymmetry of arterial pressure on hands can be observed: arterial hypertension — on the one hand and the lowered arterial pressure (or even lack of tones of Korotkov) — with another.
At occlusion of renal arteries (are possible one - and bilateral defeats) there is a syndrome of arterial hypertension in which pathogeny major importance give to activation a renin-angiotenzinovoy of system (renovascular hypertensia).
At defeat mesenteric and celiac arteries the syndrome of abdominal ischemia which is shown by abdominal pains (usually at digestion height), intestines dysfunction, weight loss of the patient is observed. At defeat of bifurcation of an aorta the syndrome of the high alternating lameness is observed — when walking there are pains in buttocks and muscles of a hip.
At damage of a pulmonary artery there are stethalgias, cough, a pneumorrhagia, an asthma, i.e. the manifestations reminding TELA. However the reason of the observed symptoms as believe, most likely, local fibrinferments in system of a pulmonary artery are.
At Takayasu's illness it is also necessary to allocate a syndrome of damage of heart which is characterized by inflammatory changes of coronary arteries with development of stenocardia, and in certain cases — a myocardial infarction. At defeat of a root of an aorta there is an expansion of an aortal ring that leads to aortal regurgitation; myocarditis with the subsequent fibrosis of a myocardium is possible; focal (cicatricial) changes in a myocardium connect with the myocardial infarction postponed earlier against a coronary vasculitis.
With Takayasu's illness quite often find a hypertrophy and dilatation of a myocardium in patients — result of process of remodeling of a myocardium against arterial hypertension, coronary insufficiency, aortal regurgitation and inflammatory changes in a cardiac muscle. As a result of all these processes at patients heart failure develops.
The course of a disease is characterized by gradual subsiding of inflammatory manifestations (though they can be resumed — a wavy current) and transition to a chronic phase with a picture of the accruing occlusion of branches of an aorta. Duration of this phase at considerable number of patients makes 10 and more than flying; it is much less at the patients having a retinopathy, aortal regurgitation or an aortic aneurysm and also at patients with a resistant inflammatory syndrome, in particular, than the accelerated SOE. The stroke, a myocardial infarction, thromboembolisms, a heart or renal failure, a rupture of aneurism can be a cause of death.
Treatment of an illness of Takayasu in an acute phase and during subsiding of the inflammatory phenomena consists in purpose of glucocorticoids and tsitostatik (Cyclophosphanum, a methotrexate). If necessary carry out the operational treatment directed to recovery of blood supply of bodies to a chronic phase of a disease — prosthetics or shunting of vessels; in certain cases balloon angioplasty with stenting of vessels is applied to recovery of a blood-groove. There are supervision showing that prolonged treatment by anticoagulants can warn fibrinferment and full occlusion of arteries at such patients.
For reference use:
The m of Takayasu (M. Takayasu) is the Japanese ophthalmologist who in 1908 described at the patient of 21 years of change of an eyeground and a type of an arteriovenous anastomosis and microaneurysms; in the subsequent messages it is noted that at patients with similar changes of an eyeground pulse often is not probed.