Clinical manifestations
Systemic symptoms A few patients may have general malaise, fatigue, fever, loss of appetite, nausea, sweating, weight loss, myalgia, arthritis and erythema nodosum a few weeks before the appearance of local symptoms or signs, which may be acute or insidious. When local symptoms or signs appear, systemic symptoms may gradually decrease or disappear, and some patients have no such symptoms.
2.Local symptoms and signs According to the affected blood vessels, there are different symptoms and signs of organ ischemia, such as headache, dizziness, syncope, stroke, vision loss, intermittent activity fatigue of limbs, weakening or disappearance of brachial or femoral artery pulsation, vascular murmur in the neck, upper and lower clavicular region, upper abdomen and kidney region, and systolic pressure difference between the two upper limbs is more than 10 mmHg.
3.Clinical typing According to the lesion site, there are four types: head and brachial artery type (aortic arch syndrome); thoracic and abdominal aorta type; extensive type and pulmonary artery type.
4.Laboratory tests There are no specific blood test items. (1) Erythrocyte sedimentation rate is an important indicator to reflect the activity of the disease. The sedimentation rate increases when the disease is active and returns to normal when the disease is stable. (2) C-reactive protein, which has the same clinical significance as sedimentation, is one of the indicators of the disease activity. (3) The increase of anti-streptococcal hemolysin “O” antibody only indicates that the patient had a recent hemolytic streptococcal infection, and only a small number of patients have a positive reaction in this disease. (4) Anti-tuberculin test Our data suggest that about 40% of patients have active tuberculosis and should be treated with anti-tuberculosis if active foci are found. Patients with a strong positive reaction to tuberculin should be examined carefully, and if the possibility of tuberculosis is confirmed, they should also be treated with anti-tuberculosis. (5) Others A few patients have increased white blood cells or increased platelets during the active phase of the disease, also as a reaction to inflammatory activity. Chronic mild anemia may occur, and hyperimmunoglobulinemia is relatively uncommon.
Imaging (1) Color multispectral ultrasonography The aorta and its major branches can be investigated for stenosis or occlusion (carotid artery, subclavian artery, renal artery, etc.), but it is more difficult to investigate its distal branches. (2) Angiography ① Digital subtraction angiography (DSA) is a digital image processing system, which is a better screening method. The advantages of this method are easy operation, short examination time, low patient burden, high contrast resolution, and the ability to show lesions in low-contrast areas. The disadvantage of this method is that the small arteries in the organs, such as the branches of the small arteries in the kidneys, are not clearly shown, and selective arteriography is still needed when necessary. (2) Arteriography can directly show the changes in the lumen of the involved vessels, the size of the diameter, whether the walls are smooth, the extent of the affected vessels and the length of the involved vessels. (3) Electronic computed scan (CT) Especially enhanced CT can show some of the lesions of the involved vessels, especially advanced CT machines and MRI can show the edema of the walls of the involved vessels to help determine whether the disease is active.
Diagnostic points]
The diagnosis is not difficult for those with typical clinical manifestations, and the disease should be suspected in women under 40 years of age with one or more of the following manifestations. (1) Unilateral or bilateral ischemic symptoms in the extremities, such as weakened or absent arterial pulses, reduced or undetectable blood pressure. (2) Ischemic symptoms in the cerebral arteries, as evidenced by weak or absent unilateral or bilateral carotid artery pulsations and cervical vascular murmurs. (3) Recent onset of hypertension or intractable hypertension with a high pitched vascular murmur above the second degree in the upper abdomen. (4) Unexplained hypothermia, vascular murmurs on both sides of the spine in the back, or in the parasternal and paramedian areas or in the renal area, and abnormal changes in the pulse. (5) Pulseless and those with fundus lesions.
2.Diagnostic criteria Adopted the classification criteria of the American College of Rheumatology in 1990.
(1) Age at onset ≤ 40 years Age at onset of symptoms or signs 10 mmHg Differential systolic blood pressure of bilateral upper limbs > 10 mmHg.
(5) Subclavian artery or aortic murmur A murmur is heard in the subclavian artery or abdominal aorta either unilaterally or bilaterally.
(6) Arteriographic abnormality Stenosis or occlusion of aortic primary branches or large arteries proximal to the upper and lower extremities, often focal or segmental in nature and not caused by atherosclerosis, fibromuscular dysplasia or similar causes. The disease is diagnosed if three of the six items above are met. It is mainly differentiated from congenital aortic stenosis, atherosclerosis, thrombo-occlusive vasculitis, leukoaraiosis, and polyarteritis nodosa.
3.Differential diagnosis
(1) Congenital aortic stenosis Most often seen in males, with high vascular murmur location, limited to the precordial region and back, no systemic manifestations of inflammatory activity, and stenosis of the thoracic aorta at specific sites (infants at the aortic isthmus, adult type at the arterial duct junction) (2) Atherosclerosis Often develops after 50 years of age, with other clinical manifestations of atherosclerosis, digital and angiography can help differentiate. (3) Fibromuscular dysplasia of the renal arteries Most commonly seen in women, with stenosis of the distal 2/3 and branches of the renal arteries on angiography, without signs of aortitis. (4) Thrombo-occlusive vasculitis (Buerger’s disease) is a chronic peripheral vascular occlusive inflammatory disease that occurs in young men with a history of smoking. It mainly involves small and medium-sized arteries and veins in the extremities, and is more common in the lower extremities. The manifestations are limb ischemia, severe pain, intermittent claudication, diminished or absent dorsalis pedis artery pulsation, wandering superficial arteritis, and in severe cases, ulceration or necrosis of the extremities, etc. It is generally not difficult to differentiate from aortitis. (5) Polyarteritis nodosa mainly involves small and medium-sized visceral arteries. The presentation is different from that of aortitis. (6) Thoracic outlet syndrome There may be diminished pulsation of the radial artery, and the pulsation changes with head and neck and upper extremity activities, and there is often venous stagnation in the upper extremity and neuropathy caused by compression of the brachial plexus nerve.
Treatment plan and principles
1.About 20% are self-limiting, and the disease is stable at the time of detection, so such patients can be followed up and observed if there are no comorbidities. For the presence of upper respiratory tract, lung or other organ infection factors in the early stage of the disease, effective infection control should be performed, which may be of some significance to prevent the development of the disease. Those with high suspicion of tuberculosis infection should be treated with concurrent anti-tuberculosis therapy.
2. Adrenocorticosteroids Hormones are still the main therapeutic drugs for the activity of the disease, and timely medication can effectively improve symptoms and relieve the disease. Generally oral prednisone 1mg/kg per day, morning dose or divided dose, maintain 3~4 weeks and then gradually reduce the dose, every 10~15 days to reduce the total amount of 5%~10%, to the blood sedimentation and C-reactive protein drop to normal as the indicator of reduction, the dose reduced to 5mg~10mg per day, should be maintained for a long time. If the conventional dose of prednisone is ineffective, it can be changed to other agents, and even high-dose intravenous shock therapy can be used in critical cases, but attention should be paid to the adverse reactions caused by hormones such as Cushing’s syndrome, susceptibility to infection, secondary hypertension, diabetes, psychiatric symptoms and gastrointestinal bleeding, etc. Long-term use should prevent osteoporosis.
3, immunosuppressants Adrenocorticotropic hormone alone is not effective, or to increase the efficacy and reduce the amount of hormone available immunosuppressants, the most commonly used drugs: cyclophosphamide, azathioprine and methotrexate. In critically ill patients, cyclophosphamide and azathioprine are administered at 2 mg to 3 mg/Kg daily, and cyclophosphamide can be given as shock therapy at 0.5 to 1.0 g/M2 body surface area every 4 weeks. Methotrexate 5mg~25mg per week can be given intravenously, intramuscularly and orally. Newer generation immunosuppressive agents such as: cyclosporine A, primaquine and leflunomide have not been reported in large clinical samples and their efficacy has yet to be confirmed. Severe cases can be life-threatening and cause major health hazards. It is now mostly believed that once aortitis is diagnosed, a combination of immunosuppressive agents and hormones should be actively started early. Even if the clinical remission, immunosuppressant maintenance use should continue for a long time, to pay attention to adverse drug reactions.
4. Improve blood circulation by vasodilating and anticoagulating drugs. The use of vasodilating and anticoagulating drugs can partially improve some clinical symptoms in patients with more obvious vascular stenosis, such as: Dibazol 20mg, 3 times a day; Tolazoline 25mg~50mg, Aspirin 75mg~100mg, 1 time a day, Disulfiram (Pansentin) 25mg, 3 times a day, etc. Blood pressure should be actively controlled in patients with high blood pressure.
5.Percutaneous endoluminal angioplasty has opened up a new way for the treatment of aortitis, and has been applied to treat renal artery stenosis and abdominal aorta, subclavian artery stenosis, etc., with better efficacy.
6.Surgical treatment The purpose of surgery is to solve renal vascular hypertension and cerebral ischemia.
(1) In cases of severe cerebral ischemia or obvious visual impairment caused by unilateral or bilateral carotid stenosis, artificial revascularization of the aorta and carotid artery, endothelial thrombectomy or cervical sympathectomy are feasible. (2) For severe stenosis of the thoracic or abdominal aorta, artificial revascularization is feasible. (3) For unilateral or bilateral renal artery stenosis, renal autotransplantation or revascularization is feasible, and nephrectomy is feasible for obvious atrophy of the affected kidney. (4) In cases of carotid sinus hyperreflexia causing recurrent syncope, carotid body removal and carotid sinus neurectomy are feasible. (5) Coronary artery bypass grafting or stenting is feasible for coronary artery stenosis.