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Sunday, April 22, 2012

Takayasu Arteritis

Takayasu arteritis-What is Takayasu arteritis?

Takayasu Arteritis, also known as high-security artery arteritis refers to the aorta and its main branches and the pulmonary artery of chronic non-specific inflammation to cause stenosis or occlusion of the different parts of the main arteritis prevalence in all regions of the world,Asia is more common, while Western European countries is rare, more common in young women, the male to female ratio 1:3.2. Arteritis of the aorta and its main branches and pulmonary chronic non-specific inflammatory changes. Mainly caused by different parts of the stenosis or occlusion, a small number of patients due to inflammatory destruction of the middle of the arterial wall may cause arterial dilatation or aneurysm. Different parts of the lesion, its clinical manifestations are different.
Takayasu arteritis - etiology and pathogenesisThe cause of Takayasu arteritis are not clear so far is generally believed that may be caused by the following factors.
Takayasu arteritis - Video (1) infection: possible pathogenic Mycobacterium tuberculosis, streptococcus, Leptospira, and Treponema pallidum and rheumatism. Infection of the blood vessel wall caused by allergic reactions or trigger the autoimmune response. Reported that this illness can have pulmonary or extrapulmonary tuberculosis, especially tuberculosis periarterial and para-aortic, 48% of patients have a history of tuberculosis, 86% in patients with positive tuberculin test. Granulomatous pathology similar to tuberculosis damage of Langerhans cells.(2) autoimmune response: In the early and active stage of the disease, often the limbs, joints, muscle pain, fever, rheumatic-like performance, erythrocyte sedimentation rate increased (stable condition erythrocyte sedimentation rate returned to normal), blood levels of gamma globulin values and the anti-aorta antibody titer increased anti-chain "O" titer, effective application of adrenal corticosteroids. Most scholars believe that this disease is an autoimmune disease.


Takayasu arteritis (3) estrogen: The disease mostly occurs in young women because young women are at the peak of secretion of various hormones, experiments prove that the bimodal pattern of estrogen secretion in the menstrual cycle of the disease patients disappears, the follicular phase estrogen hormone total is significantly higher than normal women, estrogen can significantly reduce the activity of arterial wall glycogenolysis, so that the artery wall damage. This may be related to estrogen caused by arterial smooth muscle atrophy, leading to vascular inflammation, so that the involved vessels of endometrial thickening of the fibrous tissue, thickening or thinning of the membrane, fibrosis, fibrous tissue and elastic fibers break, overlap or disappear . That estrogen caused the medial lower enzyme activity, the mechanism of inflammatory changes in the arterial wall.


(4) genetic factors: Japanese research data shows that since 1970, a total of 10 pairs of close relatives such as sisters, mother and daughter with the disease, and studies have shown that the disease and the HLA system BW40 BE52 locus, HLA-D closely related, the case of dominant inheritance. Therefore, that genetic factors in Takayasu arteritis.Takayasu arteritis - pathological changesThe vascular lesions of Takayasu arteritis was gray, wall stiffness, calcification, and atrophy with surrounding adhesions, the intimal had lead to stenosis, occlusion or thrombosis. The disease mainly affects large and medium-sized arteries, while the small and medium-sized arteries of the limbs few lesions occur. Full-thickness lesions, often involving the artery, middle degeneration and necrosis, associated with connective tissue hyperplasia and elastic fiber degeneration, the outer damage to the blood vessel wall is weak and the formation of granulomas and cystic degeneration or aneurysm.

Takayasu's arteritis refers to inflammation of the aorta and its branches. Aortitis, including syphilitic aortitis, high security arteritis, rheumatic aortitis and rheumatoid aortitis, the reason is different, but the pathological changes in eye view of the histological changes are non-specific, and are quite similar, and therefore can not simply from the naked eye changes and histological to determine the cause.

(A) syphilitic aortitisSyphilis is a sexually transmitted disease caused by Treponema pallidum. Syphilitic aortitis seen in the third period of syphilis, often more than ten years after primary infection. The lesion is characterized by aortic feeding vessels surrounded by lymphocytes, plasma cells and mononuclear cell infiltration. In the film kind of swollen gums formation of miliary and visible focal necrosis and elastic lamina damage. Late the formation of the majority of small scar. The endometrial you see a high degree of fibrosis. Eye view of the aortic intimal surface was typical of the bark-like appearance.

(B) An arteritisHigh security arteritis (Takayasu'sarteritis), also known as idiopathic aortitis (idiopathicaortitis), pulseless disease (pulselessdisease), more common in Eastern countries, but around the world have occurred
Takayasu arteritis - disease type, About 3/4 patients in adolescents, Takayasu disease, the activity of still systemic symptoms such as fever, general malaise, loss of appetite, weight loss, night sweats, joint pain and fatigue, artery lesions may have limitations pain and tenderness.2, the lesion can be divided into four types:Lesions can be divided into four types: brachiocephalic artery type (aortic arch syndrome); main renal artery type (thoracic and abdominal aortic type); hybrid (wide type); pulmonary type.

(1) of the brachiocephalic artery type (aortic arch syndrome): The lesions mainly involving the aorta and its branches. Cause varying degrees of cerebral ischemia due to carotid or vertebral artery stenosis or occlusion may have dizziness, vertigo, headache, visual disturbances, blurred vision or loss of vision, when stand up to walk or photophobia, diplopia, black spots and cataract. In severe cases, cause convulsions, aphasia, hemiplegia or coma. Head and face ischemia can cause nasal septum perforation, ulcers of the upper jaw or ear shell, teeth, chewing weakness and facial atrophy, unilateral or bilateral arterial pulse weakened or disappeared. The upper limb blood pressure was significantly reduced or undetectable and lower limb blood pressure is normal or increased. Narrow corresponding parts of vascular murmurs can be heard, especially in the neck or clavicle and sternoclavicular papillary muscle outside the triangle vascular murmur loudest accompanied by tremor. Fundus examination showed ipsilateral optic nerve head pallor, retinal arteriovenous expansion consistent with each other, surrounded by flower ring around the optic nerve head was, said high security fundus of this type because of side circulation, extremities rarely gangrene. This type accounts for about 23% -33.3%.

(2) the main renal artery type (thoracic and abdominal aortic type): due to ischemia, lower extremity weakness, pain, skin cold and intermittent claudication symptoms, especially the most obvious symptoms of iliac artery involvement. The involvement of the renal artery hypertension can include headache, dizziness, palpitations. Pulmonary stenosis, palpitations, shortness of breath, a small number of patients with angina pectoris or myocardial infarction. Hypertension-oriented type of an important clinical manifestations, especially in diastolic blood pressure increased significantly, the main renal artery stenosis caused by renal vascular hypertension; In addition, severe stenosis of the descending thoracic aorta, cardiac blood most of the flow of upper limb can cause segmental hypertension; aortic insufficiency due to systolic hypertension.

Arteritis - simple renal vascular hypertension in contrast, the lower limb systolic blood pressure than the upper limb 20 ~ 40mmHg. Or back of the spine on both sides could be heard in some patients parasternal systolic vascular murmur, murmur parts helps determine aortic stenosis location and scope, such as severe stenosis of the thoracic aorta in the chest wall, visible superficial arterial pulse, high blood pressure, upper limb in the lower limbs. About 80% of patients in the upper abdomen could be heard more than two high-profile systolic vascular murmur. Such as aortic valve insufficiency, aortic valve area could be heard and the diastolic murmur hair samples. This type accounts for about 34.8%.(3) mixed type (broad type): more common. Characteristics with the above two type of change, extensive lesions, multiple parts, the condition is generally heavier. This type is generally the first type of symptoms, with the progression of the disease evolved as a hybrid, accounting for 31.6% -41.5%.

(4) pulmonary type: the disease with pulmonary involvement is not uncommon, accounting for about 50%, these three types can be combined with pulmonary involvement, with or without pulmonary involvement, no significant difference between the various types of simple pulmonary involvement is rare. This type of pulmonary hypertension syndrome, mostly as a late complication, accounting for about 1/4, mostly mild or moderate, and severe is rare. Clinical palpitations, shortness of breath more. Severe cases, heart failure, pulmonary valve area could be heard and the systolic murmur and pulmonary valve tone hyperthyroidism.

Takayasu arteritis - clinical manifestations(A) systemic symptoms: a few weeks before the local symptoms or signs appear, the small number of patients can have symptoms of general malaise, fatigue, fever, loss of appetite, nausea, sweating, weight loss, muscle pain, arthritis and nodular erythema , acute, and may conceal the onset. When the local symptoms or signs appear, the systemic symptoms can gradually reduce or disappear, some patients had no symptoms.

(2) local signs and symptoms: According to the involvement of different vascular symptoms and signs of organ ischemia, such as headache, dizziness, syncope, stroke, vision loss, limbs activities intermittent fatigue, weakening or disappearance of the brachial artery or femoral pulse , neck, collarbone, upper and lower areas on the abdomen, kidney area appears vascular murmur, two upper extremity systolic pressure gradient greater than 10mmHg.

Takayasu arteritis - differential diagnosisTakayasu arteritis - diagnostic laboratory tests(1), blood: mild fairy blood, white blood cells increased.(2), ESR.(3), C-reactive protein is positive.(4), serum gamma globulin.(5), serum anti-aorta antibody: antibody disease ≥: 32.2 special examination(1) X-ray examination: chest X-ray examination shows the left ventricle increases, if violations of the thoracic aorta can be seen protruding aortic arch, expansion, or even tumor-like expansion, or descending aorta tapering received and pulse weakened and changed.(2) angiography: vascular edge of the visible aorta and its branches affected area irregular, with narrow and narrow expansion, aneurysm formation, or even occlusion.(3) Doppler flow imaging: exploration stenosis or occlusion of the aorta and its major branches (such as carotid artery, subclavian artery, renal artery).(3) congenital coarctation of the aorta is more common in men, a higher vascular murmur location, limited to precordial and back, systemic inflammatory activity of the performance of the thoracic aorta to see specific parts of the (baby adult in the arterial catheter in the aortic isthmus, phase at) stenosis.(4) of atherosclerotic disease, often after the age of 50 with other clinical manifestations of atherosclerosis, digital angiography can help identify.(5) renal artery fibromuscular dysplasia is more common in women, renal artery angiography showed 2/3 of its remote and branch stenosis, Takayasu's performance.(6) thromboangiitis obliterans (for Buerger disease) occurs in the smoking history of the young men around chronic vascular occlusive inflammation. Mainly involving the limbs, small and medium-sized arteries and veins, lower extremity is more common. Reduced or lost, migratory superficial arteritis, can be severe acral ulcers or necrosis, is not difficult to identify with Takayasu's arteritis generally manifested as limb ischemia, pain, intermittent claudication, the dorsalis pedis artery pulse.Takayasu arteritis - CT slices (7) nodules nodosa visceral involvement of small and medium-sized arteries. With Takayasu performance.(8) thoracic outlet syndrome in the radial artery pulse weakened, with the head, neck and upper limb activities pulsating change, and often can be caused by the phenomenon of upper extremity venous flow retention and brachial plexus compression neuropathy, neck X-ray phase show cervical rib deformities.

Diagnostic criteria1990 American Rheumatism Association classification criteria:(1), age of onset is 40 years of age.(2), intermittent claudication.(3), brachial artery pulse weakened.(4), two upper limb systolic blood pressure difference greater than 2.67 kPa (20mmHg).(5), the aortic connection Ou se of the subclavian artery vascular murmur.(6), abnormal arteriography.Where 6 3 in line with the diagnosis of the disease
Takayasu arteritis - treatment optionsTakayasu arteritis - treatment of the disease by about 20 percent are self-limiting disease has been found stable. The early onset of upper respiratory tract, lung or other organ infection factors, should be an effective infection control to prevent the development of the disease may have some significance. Highly suspected of TB infection, should also be anti-TB treatment. Commonly used drugs with glucocorticoids and immunosuppressive agents, treatment with other systemic vasculitis treatment.

 
1, the glucocorticoidHormones on the activity of the disease is still the main therapy, and timely medication can be effective in improving symptoms, remission of disease. General oral prednisone 1mg/kg daily, morning, Dayton clothing or divided doses, to maintain 3 to 4 weeks after tapering, every 10-15 days less the total amount of 5% -10%, usually to the erythrocyte sedimentation rate and C- reactive protein decline tends to be normal reduction targets, the dose to the daily 5-10mg should be long-term maintenance for some time. Such as conventional-dose prednisone does not work, switch to other formulations, the critically ill can be high-dose intravenous methylprednisolone pulse therapy. But pay attention to the hormone-induced Cushing's syndrome susceptible to infection, hypertension, diabetes, psychiatric symptoms, and gastrointestinal bleeding secondary to adverse reactions, long-term use to prevent osteoporosis.

2, the immunosuppressantsImmunosuppressants and glucocorticoids in combination can enhance the efficacy. The most commonly used immunosuppressant cyclophosphamide, azathioprine, and methotrexate. Critically ill patients with cyclophosphamide and azathioprine daily 2 ~ 3mg/kg, cyclophosphamide pulse therapy, every 3 to 4 weeks 0.5 1.0g/m2 body surface area. 5 ~ 25mg weekly methotrexate, intravenous or intramuscular injection or orally. A new generation of immunosuppressants such as cyclosporin A, mycophenolate mofetil, leflunomide efficacy remains to be confirmed. Immunosuppressive agents should pay attention to check blood, urine, and liver and kidney function in order to prevent adverse reactions appear (refer to other relevant chapters).

3, vasodilator anticoagulant to improve blood circulationThe use of vasodilators, anticoagulant therapy, part of the improvement due to vascular stenosis more obvious clinical symptoms, such as methimazole 20mg, 3 times a day; Torah cefazolin 25 ~ 50mg aspirin 75 ~ 100mg 1 day, dipyridamole (dipyridamole) 25mg daily three inferior. Active control of blood pressure in patients with hypertension.

4, percutaneous endovascular angioplastyOpened up a new avenue for the treatment of Takayasu arteritis, the current has been applied to the treatment of renal artery stenosis and abdominal aorta, subclavian artery stenosis, to obtain a good effect.

Aortitis - surgical treatment, surgical treatmentThe surgery The main purpose is to solve the renal vascular hypertension and cerebral ischemia.(1) unilateral or bilateral carotid stenosis due to severe ischemia of the brain or vision was impaired, viable artificial blood vessels of the aortic and carotid artery reconstruction, intimal thrombus enucleation or cervical sympathetic nerve resection.(2) severe stenosis of the thoracic or abdominal aorta, viable artificial blood vessel reconstruction.(3) unilateral or bilateral renal artery stenosis, viable kidney grafting or revascularization, marked atrophy of the ipsilateral kidney feasible nephrectomy.(4) of the carotid sinus hyperreflexia causing recurrent syncope hair feasible carotid body removal surgery and carotid sinus nerve resection.(5) coronary artery stenosis and coronary artery bypass surgery or stent placement.


Takayasu arteritis - prognosisTakayasu arteritis is a chronic vascular disease, involvement of the arteries due to collateral circulation, the prognosis is good, so most patients can participate in light work. Prognosis depends on the degree of hypertension and cerebral blood glucocorticoid immunosuppressant aggressive treatment can improve the prognosis. Its complications, cerebral hemorrhage, cerebral thrombosis, heart failure, renal failure, myocardial infarction, aortic insufficiency, blindness. The main causes of death as cerebral hemorrhage, renal failure.