Takayasu's disease - a systemic disease associated with granulomatous lesions of elastic aorta and its main branches.Takayasu's disease is a nonspecific aortoarteriit.The disease is related to systemic vasculitis, which are characterized by inflammatory lesions of the vascular wall with an immune component. in the pathological process may also be involved in pulmonary artery, subclavian artery, carotid artery, iliac and femoral arteries with the occurrence of the relevant symptoms.
Inflammation Takayasu's disease leads to obliteration of the lumen of the blood vessels and slow blood flow or complete halt to its authorities.Takayasu's disease is quite rare, about 2.6 cases per 1 million population per year.This lesion is more prevalent in women, and they get sick at 8 p.more often than men.
Takayasu's disease is more common in children than in adults.Suffer girls aged 11-20 years.The area of distribution - Asia, South America, Europe, in rare cases.
Takayasu disease cause
So far, the cause of Takayasu's disease is not entirely clear.Some scientists have linked the occurrence of this disease with Mycobacterium tuberculosis.The immunological reaction that is caused by bacterial antigens, is autoimmune in nature.As a result, there are antibodies to its own tissues, in particular to tissues of the arterial wall.So there is inflammation.
Takayasu's disease in the walls of the major arteries of elastic type accumulates a large number of monoliths - immune system cells.This promotes the formation of granulomas.Thus arterial disease in organs for Takayasu's disease is not typical.It was noted that in patients with nonspecific aortoarteritis a number of genes responsible for the inheritance of susceptibility to the disease.
The variant of the disease Takayasu by type sclerotic changes.In this case the loss of all sectors of the arteries and high activity of the immune system.When Takayasu disease remission at the site of inflammation formed sclerosis of the arterial walls.
Takayasu's disease symptoms
Home Takayasu's disease is usually acute.The main symptoms of the disease substrate Takayasu are immune reaction and narrowing of the blood vessels.It affects only the main outflows from the aorta.In thoracic aortic segment is carotid arteries, brachiocephalic trunk, the subclavian artery.The abdominal aorta are involved in the pathological process of renal, celiac, superior and inferior mesenteric arteries.The variant of the disease Takayasu with the complete defeat of the aorta to all trunks.Aortoarteriit also can be combined with pulmonary artery arteritis.In most cases (65%) met the clinical version with the defeat of the aortic arch and descending part of it, or mixed type.
Regardless of the localization of inflammation, Takayasu's disease starts with a high fever.There is a general fatigue and weakness, sore muscles, joints, the patient complains of a lack of appetite, weight reduction.In the initial stages it is formed with a predominance of arthritis joint damage of hands.Appears Raynaud's syndrome, which is characterized by spasm of the small blood vessels of hands.It appears on the skin sores.Can form the defeat of the pericardium, the eyeball.
Late manifestations of the disease are caused by Takayasu localization of vascular inflammation.With involvement of the subclavian arteries are affected upper limb.There are myalgia, weakness in the hands, coldness, lack of heartbeat or weak pulse in the arteries of the hands.Since unevenly affected artery, blood pressure on the hands can have different values, up to 10 units.
When the carotid artery is affected, there is a brain symptoms of ischemic stroke, cerebral infarction, ischemic dementia.From carotid artery departs vessel that feeds the eyeball, so blindness can occur.When the feeling of the neck in the carotid arteries, the patient experiences pain.
Occlusion of renal arteries leads to symptomatic hypertension.Circulating in the blood increases the products of protein metabolism, which must be removed in the urine.This option can result in Takayasu disease is lethal, due to the development of renal failure.
With involvement of pulmonary inflammation manifested pulmonary hypertension.This clinical variant leads to right ventricular heart failure, the emergence of exudate in the cavity of the body, peripheral edema, increase in liver.
in the pathological process may also be involved initial parts of the coronary arteries.The result is an occlusion of the lumen and myocardial ischemia.It may be a myocardial infarction.If developing aortic defect, then eventually develop heart failure.
Another important feature of Takayasu's disease - is intermittent claudication.This symptom is typical for the defeat of the iliac and femoral arteries.The patient thus losing the ability to walk fast.If you pick up the pace of walking, there is a sensation of pain in the muscles, numbness, weakness in the lower limbs.
With the defeat of mesenteric arteries occurs bowel ischemia.It can manifest as acute surgical pathology and chronic ischemia.In acute variant urgent intervention as necrotizing intestines lead to peritonitis and death of the patient.Symptoms of chronic ischemia is malabsorption of water and nutrients from food.The patient complains of bloating, heaviness in the stomach, nagging pains.Man loses weight, the skin becomes dry, there Zayed, the nails become thin, hair falls out.
Today noted three variants of the disease Takayasu.Acute during manifested by fever, joint syndrome, high erythrocyte sedimentation rate, anemia.Signs of ischemia occur during the 1st year of the disease.Treatment in this case ineffective.
Subacute option flow develops gradually.Low-grade temperature, symptoms of ischemia appear gradually.Changes in the analysis are expressed not as bright as in the acute form.
Chronic occur in patients older than 30 years.Vascular injury and ischemia prevail over the symptoms of intoxication.
To diagnose the disease in addition to the above symptoms Takayasu necessary to angiographic and ultrasound study of the arteries.
Takayasu's disease treatment
Treatment of Takayasu's disease must solve several problems: reduce immune inflammation, anti-ischemia, symptomatic treatment of hypertension.
In acute disease Takayasu flows must take a daily dose of prednisolone 30-40mg to reduce clinical signs.In the future, you need to reduce the dose of 5-10 mg per day.In the absence of clinical remission Treatment added methotrexate at a dose of 15 mg per day.If this therapy does not produce results, then Cyclophosphamide administered in a daily dose of 2 mg for two years.Azotioprin - 50-75 mg daily dose for 1 year
subacute disease Takayasu requires the appointment of lower doses of drugs.-20-30 Mg of prednisolone.
as prevention of thrombotic complications of Takayasu's disease take heparin, aspirin, clopidogrel, Glockenspiel.To improve peripheral blood flow and should be used Pentoxifylline ksantinola nicotinate.When high blood pressure is expedient to appoint a angiotensin-converting enzyme, if not immediately impressed two arteries.You can assign as calcium channel blockers, beta-blockers.Hemodialysis is used in the development of renal failure.
Treatment of heart failure disease Takayasu begins with the appointment of diuretics.Lasix at an initial dose of 20-30 mg, subsequently increasing the dose can.Appointed by cardiac glycosides, beta-blockers.Correct exercise and diet.Reduce consumption of salt.
resorting to surgery occlusion of the carotid and coronary arteries.Damaged blood vessels are replaced by special prostheses.Spend coronary artery bypass grafting.
Treatment of Takayasu's disease does not lead to recovery.Even with timely and adequate treatment of life is extended only for 5-7 years.
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