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Content: History of the discovery of the disease definition Epidemiology Classification Etiology Pathogenesis Clinical picture case Diagnostics Treatment

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History of the discovery of the disease by Dr. Takayasu Takayasu Mikita

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In April 1908, professor of ophthalmology at the University of Kanazawa Mikito Dr. Takayasu reported a case of specific changes in the central retinal vessels in the 22-year-old woman. Her pupils were slightly dilated and light reflexes intact, found abnormalities in the retinal vessels of the eye. In April 1908, professor of ophthalmology at the University of Kanazawa Mikito Dr. Takayasu reported a case of specific changes in the central retinal vessels in the 22-year-old woman. Her pupils were slightly dilated and light reflexes intact, found abnormalities in the retinal vessels of the eye.

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Dr. Minoru Nakajima in 1921 described the disease in the following four criteria: Dr. Minoru Nakajima in 1921 described the disease in the following four criteria: (I) affecting the bilateral eyes of young women; (II) arteriovenous anastomoses around the optic disc and formation of microaneurysms in the retina; (III) reduction of complicated cataract (IV) is not palpable radial artery. He proposed to call this disease "Takayasu's disease"

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Monument Mikita Takayasu in Kanazawa University

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definition Takayasu's disease - rarely diagnosed idiopathic disease, one of the manifestations of chronic granulomatous arteritis with a primary lesion of the aorta and its main branches characterized by a tendency to recur.

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In the pathological process involved carotid, subclavian artery and anonymous, at least - pulmonary, coronary and renal arteries. In the pathological process involved carotid, subclavian artery and anonymous, at least - pulmonary, coronary and renal arteries.

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Выделяют некоторые географические особенности распространения неспецифического аортоартериита разной локализации: Выделяют некоторые географические особенности распространения неспецифического аортоартериита разной локализации:

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Классификация болезни Такаясу в зависимости от анатомии поражения:

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Современная классификация артериита Такаясу основывается на данных, получаемых в результате ангиографического исследования. В зависимости от сосуда/сосудов, вовлечённого/вовлечённых в патологический процесс, выделяют пять типов заболевания: Современная классификация артериита Такаясу основывается на данных, получаемых в результате ангиографического исследования. В зависимости от сосуда/сосудов, вовлечённого/вовлечённых в патологический процесс, выделяют пять типов заболевания: тип 1 — ветви дуги аорты; тип 2а — восходящая аорта, дуга аорты и её ветви; тип 2б — тип 2а + грудная нисходящая аорта; тип 3 — грудная нисходящая аорта, брюшная аорта и/или почечные артерии; тип 4 — брюшная аорта и/или почечные артерии; тип 5 — тип 2б + тип 4. Если в патологический процесс вовлечены лёгочные и/или венечные артерии, то к установленному типу заболевания добавляют соответствующее указание.

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Etiology The etiology of systemic vasculitis has not been studied, but traced its connection with infectious-allergic factors and autoimmune aggression. Immunological reaction, which is caused by bacteria antigens, is autoimmune in nature. The result is appear an antibody to its own tissues, in particular to tissues of the arterial walls. So arise of inflammation.

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Suggest that the damage to the smooth muscle cells mediated cytotoxic action of CD8 T-cells, infiltrating the tissue, due to spore-forming enzyme perforin and granzyme B. Suggest that the damage to the smooth muscle cells mediated cytotoxic action of CD8 T-cells, infiltrating the tissue, due to spore-forming enzyme perforin and granzyme B.

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Role of cytotoxlic damage of tissue with CD8 T cells was confirmed by the fact that some molecules HLA I class, in particular HLA-B52, expressed at patients in excess. CD8 + T cells recognize antigens when bound to HLA molecules of class. Role of cytotoxlic damage of tissue with CD8 T cells was confirmed by the fact that some molecules HLA I class, in particular HLA-B52, expressed at patients in excess. CD8 + T cells recognize antigens when bound to HLA molecules of class.

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Currently considered the most probable genetic predisposition to appearance of Takayasu disease. Patients with this syndrome often detected HLA-DR4 gene, as well as lymphocyte antigen-CF 3. Currently considered the most probable genetic predisposition to appearance of Takayasu disease. Patients with this syndrome often detected HLA-DR4 gene, as well as lymphocyte antigen-CF 3.

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Pathogenesis The disease begins with the development of inflammation in the wall of the aorta and its major branches. As the progression of the inflammatory process in the vessel walls accumulate immune complexes; Numerous micro anguishes occur inside of the vascular membrane, leading to increased sclerosis wall and thrombosis.

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In the later stages of the disease, inflammatory elements are transformed into common atherosclerotic lesions of vessels involved in the process. In the later stages of the disease, inflammatory elements are transformed into common atherosclerotic lesions of vessels involved in the process.

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Important feature of Takayasu syndromic is simultaneous presence in the vascular wall various forms and phases of the inflammatory process: the acute stage of inflammation in one area of the vessel wall is often combined with sclerotic changes in the other. Important feature of Takayasu syndromic is simultaneous presence in the vascular wall various forms and phases of the inflammatory process: the acute stage of inflammation in one area of the vessel wall is often combined with sclerotic changes in the other.

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Clinic: Clinic: * Ischemia of the upper extremities - weakness and pain in the hands * Numbness * Low exercise tolerance, * The absence of pulsation on one or both subclavian, brachial, radial artery, * Cooling brushes.

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* A clear difference in blood pressure on the affected and healthy upper limbs, * A clear difference in blood pressure on the affected and healthy upper limbs, * Higher values of blood pressure in the lower extremities.

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* Pain in the left shoulder, the neck, the left side of the chest * Pain in the left shoulder, the neck, the left side of the chest * Changed over the arteries: palpation – painfulness auscultation - noise.

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Inflammatory lesion of the vertebral and carotid arteries Takayasu's disease causes neurological symptoms: Inflammatory lesion of the vertebral and carotid arteries Takayasu's disease causes neurological symptoms: dizziness, confusion attention and memory, decreased working ability fainting.

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The defeat of the visual analyzer: blurred vision, the appearance of diplopia, The defeat of the visual analyzer: blurred vision, the appearance of diplopia, * sometimes sudden blindness in one eye due to acute occlusion of the CRC (central retinal artery) and optic nerve atrophy.

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Vision due to diplopia Vision due to diplopia

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Pathological expansion and consolidation of the ascending aorta in patients with Takayasu's disease often leads to aortic insufficiency, coronary circulatory disorders, ischemia and myocardial infarction. Pathological expansion and consolidation of the ascending aorta in patients with Takayasu's disease often leads to aortic insufficiency, coronary circulatory disorders, ischemia and myocardial infarction.

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Echograms with aortic insufficiency and aortic stenosis

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Changes in the abdominal aorta cause a progressive decrease in blood circulation in the lower extremities, pain in the legs while walking. Changes in the abdominal aorta cause a progressive decrease in blood circulation in the lower extremities, pain in the legs while walking.

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Takayasu's disease is marked articular syndrome - arthralgia Takayasu's disease is marked articular syndrome - arthralgia

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Migratory arthritis with predominant involvement of the hand joints. Migratory arthritis with predominant involvement of the hand joints.

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When kidney disease of the arteries: When kidney disease of the arteries: proteinuria, hematuria, rarely thrombosis. The involvement of the pulmonary artery manifests itself with pain in the chest, shortness of breath, development of pulmonary hypertension.

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ЭЭГ

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Результат микробиологического исследования

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International Journal of Rheumatic diseases 2014; 17: 931-935 p. Комлексное лечение вазоренальной гипертензии при неспецифическим аортоартериите Демеуов Т.Н. Национальный научный центр хирургии им. А.Н. Сызганова УДК: 616. 13- 008.331.1-089