I. The concept of polyarteritis major
Polyarteritis major, also known as Takayasu disease, pulseless disease, aortic arch syndrome, etc., is a relatively common primary immune chronic inflammatory arterial disease. It occurs mostly in adolescent females, with a male to female ratio of 1:8, and the age of onset is mostly 20 to 30 years. The disease is characterized by multiple, nonsuppurative inflammatory diseases of the aorta and its major branches, often involving several vessels, resulting in stenosis or occlusion of the affected vessels, and rarely causing dilatation or aneurysm formation.
Multiple aortitis belongs to the category of “pulse paralysis”, “blood paralysis” and “vertigo” in Chinese medicine. If gangrene occurs due to severe ischemia, it falls under the category of “gangrene removal”. The “Heart of Medicine” says: “Those who do not come out of the volatile veins are also occluded by cold.”
Second, the etiology and pathogenesis
The cause of multiple aortitis is still unclear, but may be related to the following factors.
1, estrogen: the disease is common in young women, and this period is the peak of various types of female hormones, some experiments have shown that the bimodal pattern of estrogen secretion in the menstrual cycle disappears in cases of this disease, the total amount of estrogen in the follicular phase is significantly higher than normal women, and estrogen can significantly reduce the activity of glycogen breakdown in the arterial wall, so that the arterial wall is damaged.
This may be related to estrogen-induced atrophy of arterial smooth muscle, leading to an inflammatory vascular response that results in thickening of fibroblastic tissue in the intima, thickening or thinning of the intima, fibrous degeneration, and breakage, overlap, or loss of fibrous tissue and elastic fibers in the affected vessels. It is also believed that estrogen causes a decrease in the activity of certain enzymes in the arterial intima, which is the mechanism of inflammatory changes in the arterial wall.
2. Infection: The most common causative agents are Mycobacterium tuberculosis and Streptococcus. Infection causes a metamorphosis of the vessel wall or triggers an autoimmune reaction. It has been reported that patients with this disease may have concurrent pulmonary or extrapulmonary tuberculosis lesions, especially periarterial and para-aortic tuberculosis lesions. 48% of patients have a history of tuberculosis, and 86% of patients have positive tuberculin tests. Pathologically there are Langerhans cell granulomas that resemble tuberculosis damage. It has also been suggested that the disease is associated with rheumatic fever.
3. Autoimmune reaction: In the early and active stages of the disease, there are often joint and muscle pain in the limbs, low fever and other manifestations similar to rheumatism, increased blood sedimentation, increased gammaglobulin values and anti-aortic antibody potency in the blood, increased anti-chain “O” titers, and effective treatment with adrenocorticosteroids. Therefore, most scholars believe that this disease is an autoimmune disease.
Genetic factors: Since 1970, 10 pairs of close relatives, such as sisters and mothers and daughters, have been found to have this disease in Japan, and studies have shown that the disease is closely related to the BW40 and BE52 loci and HLA-D in the HLA system, which are dominantly inherited. Therefore, it is believed that polyarteritis major has a congenital genetic factor.
Multiple aortitis is caused by infection, drugs and other factors acting on the organism, causing an immune response, dysfunction of autoimmunity, making the aortic wall antigenic, and the immune active cells of the organism cannot recognize it, producing anti-aortic antibodies after binding to this antigen and acting on the aortic wall, resulting in an antigen-antibody reaction, forming immune complexes, depositing in the vessel wall, and non-specific inflammation, causing immune damage, which in turn leads to aortic stenosis and occlusion.
Pathological changes
The diseased vessels are grayish-white in color, with stiffness, calcification, atrophy and adhesion to the periphery, and narrowing or occlusion of the lumen. The disease mainly affects the large and medium-sized arteries, while the small and medium-sized arteries of the limbs rarely develop lesions. The lesions often involve the entire arterial layer, with connective tissue hyperplasia and degenerative elastic fibers, which may result in granulomas and cystic changes or aneurysm formation. The disease can be divided into head and arm type, thoracoabdominal aorta type, renal artery type, mixed type and pulmonary artery type according to the distribution of lesions invading arteries.
D. Chinese medicine etiology and pathogenesis
Chinese medicine believes that this disease is due to congenital deficiency of endowment, acquired spleen and stomach disorders, resulting in deficiency of qi and blood, and then due to the invasion of cold and dampness, resulting in damage to the pulse channels, obstruction of the meridians, Qi and blood stagnation, Qi stagnation and blood stagnation; or due to dietary disorders, damage to the spleen and stomach, loss of transport and transformation, phlegm and dampness, internal growth, blocking the pulse channels, phlegm and stagnation of the meridians; or due to the deficiency of spleen and kidney yang, unable to warm, cold stagnation of the veins; or liver and kidney yin deficiency, the tendons and veins are not moistened, the veins are astringent The pulse is paralyzed, resulting in no pulse. Many factors affect the final obstruction of the pulse pathway, the meridians do not pass and the cost of the disease.
V. Medical history
Multiple aortitis occurs in adolescent females, with a peak incidence at the age of 20 to 30 years, with chronic progressive changes and a disease duration of more than 20 years. At the beginning of the disease, there are systemic symptoms such as low fever, decreased appetite, fatigue, wandering joint pain, and increased blood sedimentation, which are often misdiagnosed as rheumatism; some patients with hypertension are seen with hypertension and gradually show ischemia in the limbs, which attracts the attention of patients, but rarely find ulcers or gangrene in the limbs.
VI. Clinical manifestations
The clinical symptoms and signs of polyarteritis depend on the site of lesion involvement and are usually classified into four types.
1. Head and arm artery type: The lesions mainly involve the aorta and its branches. Due to narrowing or occlusion of the carotid artery or vertebral artery causing different degrees of cerebral ischemia, there may be dizziness, vertigo, headache, visual impairment, blurred vision or loss of vision when getting up and walking, also shyness, diplopia, black spots, cataracts. Severe cases cause convulsions, aphasia, hemiparesis or coma. Due to ischemia of the head and face, nasal septum perforation, ulceration of the upper jaw or ear shell, loss of teeth, weakness of chewing and facial atrophy, and weakening or loss of unilateral or bilateral arterial pulsations may result.
Blood pressure in the upper extremities may be significantly reduced or undetectable, while blood pressure in the lower extremities may be normal or increased. Vascular murmurs can be heard in the corresponding areas of the stenosis, especially in the neck or supraclavicular and external triangle of the sternocleidomastoid muscle, which are the loudest with tremor. Funduscopic examination shows pale optic papillae on the affected side and dilated retinal arterioles, which anastomose with each other and surround the optic papillae in a wreath-like pattern, called Gaoan fundus. This type accounts for about 23% to 33.3%.
2. Main and renal artery type: The lesion mainly involves the descending and abdominal aorta and its branches. Weakness of the lower limbs, chills and intermittent claudication occur due to lower limb ischemia. Hypertension often occurs due to narrowing of the abdominal aorta and renal artery stenosis, and severe hypertension can lead to heart failure with palpitations, dyspnea, fatigue and weakness. Intestinal ischemia can cause colic, diarrhea, blood in stool, etc. If the lesion spreads to the aortic valve, it will cause aortic valve closure insufficiency.
If the lesion extends to the opening of coronary artery and its vicinity, it may cause angina pectoris or myocardial infarction. Physical examination may reveal weakened or absent arterial pulsations in the lower extremities and significantly reduced or undetectable blood pressure, while blood pressure in the upper extremities is significantly elevated. Depending on the location of the lesion, vascular murmurs may be heard on both sides of the parasternal or spinal column, or in the upper abdomen. If complicated by aortic valve closure insufficiency, a blowing wind-like diastolic murmur is heard in the aortic valve area, fundus examination shows hypertensive fundus changes, electrocardiogram left ventricular hypertrophy with strain, this type accounts for about 34.8%.
3, mixed type: more common. With the characteristics of the above two types of changes, extensive lesions, multiple sites, the disease is generally more serious. This type generally has the symptoms of one type first, and gradually evolves into a mixed type with the development of the disease, accounting for about 31.6% to 41.5%.
4, pulmonary artery type: the lesion mainly involves the pulmonary artery, because there is abundant collateral circulation around the artery, so the ischemic symptoms are not obvious, rarely appear respiratory symptoms, severe cases may appear palpitations, shortness of breath or intermittent hemoptysis, pulmonary valve area can be heard in the systolic murmur. It has been reported that pulmonary artery involvement in polyarteritis is as high as 14% to 50%, and patients can be asymptomatic, but 63% have pulmonary hypertension or right ventricular strain, and 5% of cases can be accompanied by pleural fluid.
Seven, auxiliary tests
1.Laboratory tests.
2.Blood test.
3.Urinal fluid examination.
4.Electrocardiogram and echocardiogram.
5.Cerebral hemogram.
6.X-ray.
VIII. Diagnostic criteria
The criteria developed by the Committee of Peripheral Vascular Diseases of the Chinese Society of Integrative Medicine in 1995 are as follows.
1, unilateral or bilateral limbs with ischemic symptoms: coldness, fear of cold, weakness of limbs mainly, accompanied by weakening or disappearance of arterial pulsation, reduced blood pressure or pulse pressure difference between two limbs >15-20 mmHg, or blood pressure of upper limbs higher than that of lower limbs.
2, head ischemic symptoms: vertigo (especially when tilting the head), fainting episodes, visual impairment, neck vascular pain with weakening or disappearance of carotid pulsation, arterial murmur heard in the neck.
3.Symptoms of intractable hypertension: headache, vertigo, chest tightness, shortness of breath, etc., and vascular murmurs of grade II or higher are heard in the periaqueductal region of the abdomen or the renal region of the lumbar region.
4.Arterial vascular murmurs are heard in the neck, supraclavicular region, back and abdomen (can be heard without pressure in the abdomen in women), with corresponding ischemic signs.
5. Systemic symptoms: fever, joint or muscle pain, lethargy, nodular erythema of skin, fast sedimentation, positive CRP, increased 7-globulin, increased anti-chain “O”, and aggravation of the original ischemic symptoms and signs during the acute phase (early stage) or re-emergence activity.
6. Typical fundus changes of Gaoan eyes.
7. Arteriography, ultrasound Doppler, ECT and other examinations prove that the affected head and arm arteries and lower limb arteries show stenosis or occlusion, and the descending aorta and abdominal aorta show constriction.
IX. Treatment methods
Method 1: Treatment period Treatment is mainly non-surgical.
1.Corticosteroids can inhibit inflammation, improve symptoms and stabilize the condition. At present, long-term oral administration of small doses of hormones is advocated, with small side effects and ideal symptom control. In addition to the use of corticosteroids, the use of gammaglobulin sometimes has a significant effect on the relief of symptoms.
2.Vasodilator drugs On the basis of controlling the development of inflammation, it can also be supplemented with vasodilator drugs such as tolazurin, 25mg each time 3 times a day, and dibazol, 100ml each time 3 times a day, to improve the ischemic symptoms. Recently, some scholars believe that the above drugs can only improve the blood flow of normal blood vessels, and the dilating effect on the narrowed blood vessels is weak, and even aggravate the distal ischemia instead. The common dose is 400mg, divided into 3-4 times, and its clinical efficacy needs to be further observed.
3, reduce blood viscosity drugs Recent studies have concluded that patients with polyarteritis have a hypercoagulable state, which provides a theoretical basis for the use of low-molecular dextrose, such as the addition of blood-stasis activating salvia, which can make the effect more obvious. This method is effective in cerebral ischemia patients. Commonly used dose is 500ml of low molecular dextrose plus 8 to 10 sticks of salvia. It is given once a day for 14 days as a course of treatment. In addition, abdominal snake antithrombin has been used clinically because it has the effect of reducing fibrinogen and reducing platelet anticoagulation.
4, anti-platelet aggregation drugs Pansentin each 25mg daily, enteric aspirin each 0.3, once a day and other drugs have inhibited platelet aggregation, can be used as adjuvant drugs. The vasodilating and anti-platelet aggregation effects of prostacyclin have been gradually recognized, but it is not yet used in this disease.
The above treatment may help to relieve the symptoms of fever, dizziness, headache, fatigue and joint pain to some extent.
Indications for non-surgical treatment in the stable phase.
①Mild lesions without significant hemodynamic changes;
②Severe vascular lesions, extensive obstruction, and poor general condition cannot tolerate surgery;
(iii) Simple upper limb pulselessness. The main purpose of treatment: to try to improve the symptoms of ischemia in the brain, kidneys and other major organs, and to control intractable hypertension.
In China, polyarteritis is a highly common cause of renal vascular hypertension. Captopil (SQ14225 methoprene) is an angiotensin-converting enzyme inhibitor that blocks the conversion of angiotensin I to angiotensin II, with a satisfactory antihypertensive effect, and the commonly used dose is 25-50 mg, 2-3 times daily.
Surgery: Patients with luminal stenosis or even occlusion, producing severe ischemia affecting function in different parts of the brain, kidneys, upper and lower limbs, and those with severe intractable hypertension for which drug treatment is ineffective, should be treated surgically. Generally, surgery should be performed six months to one year after the lesion is stabilized and when the organ function has not disappeared.
Method 2: Chinese medicine evidence-based treatment of multiple aortitis
1. Heat and toxicity blocking the ligaments (active stage)
The symptoms include body heat, muscle and joint pain, tiredness, insomnia, dry mouth and cold drinks, dry stool, yellow urine, red tongue with thin yellow coating, weak pulse or no pulse, and number of pulse.
Treatment: Clearing heat and detoxifying toxins, activating blood circulation and removing blood stasis
2.Dampness and heat stagnation evidence (active period)
The symptoms include fever or hot flashes, tiredness and heaviness, numbness of the limbs, sore joints, lack of food and drink, distention and fullness of the stomach, loose and yellow urine, red and white tongue, yellow or white greasy moss, weak or no pulse, and number of pulse.
Treatment: Clearing heat and dampness, activating blood circulation.
3.Weakness of Qi and Blood (chronic phase)
See palpitations and shortness of breath, dizziness, insomnia and dreaminess, tiredness and weakness, cool and numb limbs, soreness and pain, light tongue with thin white coating, sunken and thin pulse or no pulse, rather no pulse disease.
Treatment: Benefit Qi and tonify Blood. Activate the blood and open the channels.
4.Qi stagnation and blood stasis (chronic phase)
The symptoms include irritability, good breath, chest tightness, headache, dizziness, blurred vision, pain in the chest and back or swelling and pain in the two hypochondrium, tiredness of the limbs, numbness, insomnia and dreaminess, dark tongue or petechiae, thin pulse or no pulse.
Treatment: Diversify liver and Qi, activate blood circulation
5.Yin deficiency of liver and kidney, hyperactivity of liver and Yang (chronic phase)
The symptoms include soreness and weakness of the waist and knees, numbness of the limbs, tiredness and weakness, heat in the heart of the hands and feet, dry mouth and throat, insomnia and forgetfulness, tinnitus and deafness, dizziness and dizziness, trembling of the lower limbs, lack of warmth at the end of the four extremities, low menstrual volume and darkness or amenorrhea, red tongue or red tongue tip, little moss, thin or weak pulse or no pulse. Corresponds to renal artery stenosis or mixed type.
Treatment: Nourish Yin and submerge Yang, invigorate Blood and open the veins.
6.Spleen and kidney yang deficiency (chronic phase)
The symptoms include weakness of the waist and knees, numbness of the limbs, coldness and weakness of the limbs, abdominal fussiness and lack of energy, abdominal distension and loose stools, fear of cold and warmth, fatigue and forgetfulness, dizziness and shortness of breath, abdominal pain during menstruation, pale white face, pale tongue with fatty coating and white fur, weak pulse or no pulse, and disappearance of the Yang pulse.
Treatment: Warming the kidney and strengthening the spleen, dispersing cold and invigorating blood.