(Takayasu’s arteritis, pulseless disease, occlusive Thromboaortopathie, aortic arch syndrome)
Takayasu’s arteritis is an inflammatory disease that affects the aorta, its side branches and the pulmonary arteries. It occurs mainly in young women, its etiology is unknown. Vascular inflammation cause artery stenosis, occlusion, dilatation or aneurysm. The patients show an asymmetrical pulse state or uneven blood pressure measurements on the different sides (for. Example, between the extremities of the left and right side, or between the arm and the leg of the same page), claudication of the extremities, symptoms of decreased cerebral blood flow (eg. as transient visual disturbances, transient ischemic attack, stroke), and hypertension or its complications. The diagnosis is made by an arteriography of the aorta or a magnetic resonance angiography. The treatment consists of the administration of corticosteroids and other immunosuppressive drugs and, in organ-threatening ischemia, in vascular surgical procedures such as bypass surgery.
Takayasu’s arteritis is rare. It is more common in Asians, however, occurs worldwide. The male to female ratio is 1: 8, the age of onset is between 15 and 30 years. In North America, the annual incidence of 2.6 cases / million is. valued.
Takayasu’s arteritis is an inflammatory disease that affects the aorta, its side branches and the pulmonary arteries. It occurs mainly in young women, its etiology is unknown. Vascular inflammation cause artery stenosis, occlusion, dilatation or aneurysm. The patients show an asymmetrical pulse state or uneven blood pressure measurements on the different sides (for. Example, between the extremities of the left and right side, or between the arm and the leg of the same page), claudication of the extremities, symptoms of decreased cerebral blood flow (eg. as transient visual disturbances, transient ischemic attack, stroke), and hypertension or its complications. The diagnosis is made by an arteriography of the aorta or a magnetic resonance angiography. The treatment consists of the administration of corticosteroids and other immunosuppressive drugs and, in organ-threatening ischemia, in vascular surgical procedures such as bypass surgery. Takayasu’s arteritis is rare. It is more common in Asians, however, occurs worldwide. The male to female ratio is 1: 8, the age of onset is between 15 and 30 years. In North America, the annual incidence of 2.6 cases / million is. valued. Etiology The cause is unknown. Cell-mediated Immunmantworten may be involved. Pathophysiology Takayasu’s arteritis primarily affects large elastic arteries. They are most commonly affected unknown and collarbone artery aorta (especially the ascending aorta and the arch) common carotid artery renal arteries Most patients have stenosis or occlusion. Aneurysms occur in about one third of patients. Typically, the wall of the aorta or its branches thickened irregular, with wrinkling of the intima. If the aortic arch is concerned, the mouths of the great arteries emerging from the aorta, significantly narrowed or even obliterated due to a thickening of the intima are. the pulmonary arteries are affected in one half of the patients. Occasionally, the medium-sized branches of the pulmonary arteries are involved. Histologically, early changes in mononuclear infiltrates the adventitia with perivascular cuffing of the vasa vasorum. Later, an intense mononuclear inflammation of the media occurs, sometimes accompanied by granulomatous disease, giant cells and patchy necrosis of the media. The morphological changes can not be distinguished from those of a giant. Panarteriitische inflammatory infiltrates lead to a significant thickening of the affected artery and subsequent luminal narrowing and occlusion. Symptoms and signs Most patients show only focal symptoms that reflect the reduced circulation of the affected organ or limb affected. About a third of patients reported common symptoms such as fever, malaise, night sweats, weight loss, fatigue and / or arthralgia. Repetitive arm movements and persistent elevation of the arm lead to pain and fatigue. The arterial pulses in the arms and legs can be reduced and asymmetrical. The limbs show signs of ischemia (z. B. Cool, intermittent claudication). Often, vessel noise on the subclavian artery, the brachial artery audible, the Korotiden, the abdominal aorta or the femoral arteries. Decreased blood pressure in one or both arms is common. The involvement of the carotid artery and the vertebral artery which leads manifested by dizziness, syncope, orthostatic hypotension, headache, temporary visual impairment, transient ischemic attack or stroke at a reduced cerebral blood flow. Stenoses of the subclavian artery near the origin of the open vertebral artery can cause a posterior circulation, ischemic neurological symptoms or syncope, when the arm is used (so-called. Subclavian steal syndrome). The mechanisms are retrograde flow through the vertebral arteries to supply the subclavian artery distal to the stenosis and vasodilatation of the arterial bed in the upper extremities during training. By narrowing the mouth of the coronary arteries due to a aortitis or coronary arteritis can lead to angina or myocardial infarction. If the ascending aorta is significantly dilated aortic insufficiency occurs. Also, heart failure may develop. Sometimes an obstruction of the aorta descendens calls signs of Aortenverengung forth (hypertension, headache, intermittent claudication). If the abdominal aorta or the renal arteries are narrowed, a renovascular hypertension developed. A temporary claudication of the arm or leg can develop. It affects the pulmonary arteries, which occasionally leads to pulmonary hypertension. Participation of medium-sized branches of the pulmonary artery can cause pulmonary infarction. Since the Takayasu’s arteritis is chronic, a collateral circulation may develop. Therefore, ischemic ulcers or gangrene due to the obstruction of limb arteries are rare. Diagnostic aortography, MR angiography or CT angiography monitoring of disease activity on the diagnosis of Takayasu’s arteritis is to think if symptoms suggest an ischemia of organs that are supplied by the aorta or its branches, or if the peripheral pulses are reduced or missing in patients with a low risk of atherosclerosis and other Aortenstörungen, especially in young women. In these patients, arterial sounds and side differences of pulse and blood pressure in the arms or legs are also suspicious. Confirmation of the diagnosis requires a aortography. Alternatively, a MR angiography or CT angiography can be done to assess all branches of the aorta. Typical findings are stenoses, occlusions, arterial caliber fluctuations, post stenotic dilatation and collateral arteries in the area of ??stenosed vessels, also aneurysms. Blood pressure is measured in all limbs. However, an accurate measurement of blood pressure can be difficult. If both Aa. subclaviae are severely affected, the blood pressure can be measured only in the legs exactly. the disease affects both Aa. subclaviae and show the patient a coarctation of the ascending aorta and / or participation of the two Aa. iliac or Aa. femoral, blood pressure at any limb can be accurately measured. Then the central arterial blood pressure needs to be determined by angiography to detect occult hypertension, which can cause complications. Further evidence of an occult hypertension can give Funduscopic signs of hypertensive retinopathy and / or echocardiographic evidence of concentric left ventricular hypertrophy. If severe hypertension is not recognized, the complications with signs of vasculitis, which leads to a Organischämie can be confused. Laboratory diagnosis provides only non-specific and unhelpful results. Common findings include anemia associated with chronic disease, increased platelet count, white blood cell count and occasionally increased elevated ESR and CRP. Indicators of disease activity in Takayasu’s arteritis are: Symptoms and signs: New systemic symptoms (eg, fever, fatigue, weight loss, loss of appetite, night sweats.), Symptoms suggestive of vasculitis, including new arterial territories (eg claudication. ), new cases of vessel noise and / or changes in blood pressure laboratory tests: the detection of inflammation, which can be detected by blood tests (although the inflammation markers may also see an active arteritis) Imaging techniques: development of stenosis or aneurysms in previously unaffected arteries (assessed based on a periodic imaging [usually MR angiography]) However, Takayasu’s arteritis can progress silently, even though clinical and laboratory studies suggest a complete remission. Therefore, a periodic imaging of the aorta and large arteries is mandatory. Blood pressure should be measured regularly in an unaffected limb. Imaging techniques in Takayasu arteritis process applications Comments Conventional angiography (aortography) Preferably, when surgery is being considered and when the Bludruck the proximal aorta can not be measured in other ways Provides descriptive anatomical information on the vessel lumen MR angiography of the aorta and the great arteries prevents the risk of an arterial puncture and exposure to iodinated contrast agents or radiation Normally, the method of choice for young women who are less likely to be affected by severe atherosclerosis and are more susceptible to radiation-induced cancer Provides some information about the anatomy of the artery wall does not deliver enough information about the distal aortic branches, since the resolution is too low Provides any information about the content of arterial plaque, which is difficult makes a distinction between vasculitis and atherosclerosis CT angiography is the general study of the aorta and its proximal branches used when an MR angiography should be available contraindicated or not Can a calcification of the aorta characterize Provides also information on the arterial wall thickness Unclear whether it makes sense to monitor the disease activity positron emission tomography with fluorine-18 (18F) -Deoxyglucose is used to assess regional differences in glucose metabolism, and can help to localize inflammation regions (since inflammatory cells absorb more glucose) Provides no information on changes lumen size illnesses that mimic Takayasu’s arteritis must be excluded. These include: Inherited non-inflammatory connective tissue diseases (. Eg, Ehlers-Danlos syndrome, Marfan syndrome), vascular infection (tuberculosis, fungal or syphilitic) fibromuscular dysplasia disorders caused arterial thrombosis (eg hypercoagulable) Idiopathic inflammatory diseases (such as ankylosing spondylitis. with aortitis, RA, Cogan’s syndrome or Behcet’s syndrome, Kawasaki disease, sarcoidosis) All of these disorders can affect the large blood vessels. Prognosis of the course is monophasic in 20% of patients. For the rest of the course of relapses and remissions is characterized or chronic and progressive. Even when symptoms and laboratory abnormalities suggest a standstill, come to new vascular lesions and are detectable in imaging tests. A progressive course and the presence of complications (eg. As hypertension, aortic regurgitation, heart failure, aneurysms) indicate a poor prognosis. Corticosteroids therapy Occasionally other immunosuppressants antihypertensives and / or vascular procedures if necessary Drug therapy cornerstone of treatment of Takayasu’s arteritis are corticosteroids. The optimal dose and duration of treatment Ausschleichplan are not fixed. In most patients, treatment with corticosteroids alone leads to remission. Usually prednisone is used. The initial dose is 1 mg / kg p.o. once a day for 1-3 months; the dose is then tapered off slowly over several months. Lower starting doses may also initiate a remission. About half of the patients suffer despite initial response to relapse when the drug then tapered or discontinued. Methotrexate, cyclophosphamide, azathioprine, mycophenolate mofetil and TNF inhibitors (z. B. etanercept, infliximab) have been used successfully in some patients. They can be used when corticosteroids are not sufficiently effective or can not be tapered. The initial dose of methotrexate is 0.3 mg / kg once a week and is increased to up to 25 mg per week. Mycophenolate mofetil may also be tried. Cyclophosphamide should be considered in patients with coronary vasculitis or other serious complications, which are believed to be due to an active arteritis. A platelet inhibitor (eg. As 325 mg of aspirin po once daily) is often used as a platelet-mediated occlusion can not be excluded. When present, hypertension should be treated consistently; The use of ACE inhibitors may be useful sein.Verfahren A vascular intervention, usually a bypass procedure may be necessary in order to restore blood flow to ischemic tissues if drug therapy proves ineffective. These include the following indications: aortic stenosis of the coronary arteries that symptomatic coronary heart disease or ischemic cardiomyopathy cause dissection of an extended aortic aneurysm severe hypertension as a result of renal artery stenosis, the refractory responding to treatment ischemia of the extremities, the everyday activities affect brain ischemia coarctation inability to to measure blood pressure accurately (in all limbs) bypass surgery, preferably with autologous transplants have the best patency rates. The anastomosis should be made to not affected by the disease sites of the arteries, to prevent aneurysm formation and occlusion. Percutaneous transluminal coronary angioplasty (PTCA) has some risks and can be effective in short lesions. The rate of long-term restenosis seems here to be much higher than with bypass surgery. A vascular stent implantation is not usually recommended because the restenosis rate is high. In an aortic a flap surgery with reimplantation of the aortic root may be necessary. Summary Takayasu’s arteritis is a rare arteritis that mainly affects women aged 15-30 years. The involvement of the aorta, the pulmonary artery and its branches may cause symptoms such as asymmetric pulses or blood pressure measurements, claudication, symptoms of decreased cerebral blood flow (z. B. temporary visual impairment, transient ischemic attacks, stroke) and hypertension (systemic and pulmonary) or its complications. The diagnosis is made by MR angiography or occasionally CT or conventional angiography. The treatment consists of the administration of corticosteroids, other immunosuppressants, aspirin and, if indicated, antihypertensives. The patients have to be transferred to a vascular surgery when despite medical therapy major vascular complications (eg Endorganischämie;. Aortic dissection, -koarktation or insufficiency) show.