Aortitis occurs mainly in the main blood vessels of the body and their branches. There are four main types of aortitis: head and arm type-ischemia mainly in the head, face and upper limbs, manifested as dizziness, loss of vision or even blindness, reduced or even undetectable blood pressure in the upper limbs, and pulse may disappear. Thoracic aorta type – mainly manifests as severe hypertension in the upper body and hypotension in the lower body. Abdominal aortic type – mainly presents with hypertension due to renal artery stenosis, some patients also present with chronic abdominal pain (intestinal ischemia). Mixed type-both of the aforementioned two or more types. Treatment is based on a combination of Chinese and Western medicine and medication, and if necessary, interventional surgery.
Aortitis is an arterial disease that occurs in the aorta and/or its major branches with chronic nonspecific inflammation, causing localized intimal fibrosis, mid-layer degeneration and fragmentation, and outer membrane fibrosis, resulting in luminal narrowing or even occlusion.
The pathology of this disease is characterized by the possibility of lesions in all parts of the large arteries, often involving several vessels at the same time, mostly resulting in stenosis or occlusion of the affected vessels, and in a few cases, aneurysmal dilatation. The lesions are grayish in color, with stiff, calcified, atrophic walls, adhesions to surrounding tissue, and luminal narrowing or occlusion. In rare cases, it may lead to arterial dilatation or even aneurysm formation.
[Clinical features]
The disease mostly occurs in young people, most often in women aged 21 to 30 years.
The development of the disease is mostly slow, and the early symptoms vary in severity from low fever and pain in the chest and abdomen or neck root in mild cases to high fever and chest pain in severe cases, and a few may have a skin rash. Because such early patients are still in the pathologic stage of large vessel inflammation, no significant stenosis or occlusion has yet appeared. Therefore, during physical examination, only muscle and joint pain and pressure in the large blood vessel area can be found, but there are no obvious signs of insufficient blood supply. As the disease progresses over several years, the artery becomes stenosed or occluded due to the aggravation of the lesion, and a series of clinical manifestations of blood supply deficiency appear.
Depending on the location of the involved vessels, patients can be classified into four types: cephalobrachial, which involves the aortic arch and its main branches; thoracoabdominal, which mainly involves the descending aorta or abdominal aorta; renal artery, which involves the renal artery alone; and mixed, in which the lesion involves more than two groups of these vessels.
The clinical manifestations are described according to the types.
( I) Head and arm type
In this type of patients, the vascular lesions are in the large branches of the aortic arch, such as the common carotid artery, subclavian artery and the unnamed artery, and may involve one branch alone or all branches at the same time. Sometimes the lesions may also involve the internal carotid artery or the vertebral artery. The symptoms of this type are mainly manifested in two aspects: one is the manifestation of upper limb ischemia, and the other is the symptom of head, face and brain ischemia.
When the innominate artery or subclavian artery is involved, there are symptoms of insufficient blood supply to the upper limbs, such as cold and cold fingers, soreness and weakness, and pain after exertion. When the upper extremity ischemia there is muscle atrophy of the upper extremity, blood pressure cannot be measured or is significantly reduced, while the blood pressure of the lower extremity is still normal or increased. Because of the formation of abundant collateral circulation, necrosis of the finger ends is less likely to occur even in the late stage of the disease.
When the common carotid artery and the unnamed artery become narrowed or occluded resulting in obvious cerebral ischemic symptoms, cerebral circulation disorders can take two forms, one manifesting as chronic cerebral ischemic symptoms and the other as transient cerebral ischemic attacks. The manifestations of chronic cerebral ischemic symptoms are more diverse. Initially, there may be tinnitus and blurred vision, and some patients complain of flashing dots in the eyes and feel a white curtain in front of their eyes. Later, memory loss, drowsiness or insomnia, dreaminess, dizziness, etc. may gradually appear. In the later stage of the disease, patients often become dizzy and have difficulty standing, and even have to take the head low and foot high position when lying down. TIA is a transient interruption of cerebral blood flow, such as vertigo, momentary blackness, or in severe cases, episodic syncope or even hemiplegic coma.
On physical examination, patients with the cephalobrachial pattern may find that the arterial pulsations distal to the affected vessels are diminished or absent, such as the common carotid, temporal, axillary, or radial arteries, which may be significantly diminished or absent, manifesting as pulselessness. There may be pressure pain in the carotid artery and stiffness of the arterial wall. In addition, tremor may be felt or systolic murmur may be heard in the supraclavicular region or carotid region. The murmur of the carotid artery may be transmitted in the direction of the head, and the loudness of the murmur sometimes reaches grade 3-4.
( 2) Thoracoabdominal aortic type
In patients with thoracoabdominal aortic type, the lesions mainly occur in the thoracic or abdominal aorta, mostly resulting in stenosis or occlusion of the thoracoabdominal aorta. In this case, the peripheral vascular resistance of the heart increases significantly and the blood flow to the lower extremities decreases significantly, so the clinical manifestations are mainly insufficient blood supply to the lower extremities and hypertension of the head, neck and upper extremities, such as coldness of the lower extremities, soreness and weakness of both lower extremities after walking, intermittent claudication, etc., accompanied by dizziness, headache, palpitations, and heart failure in severe cases. In some patients with thoracoabdominal aorta, angiography and pathological examination have revealed changes of aortitis in the pulmonary artery or abdominal artery, and the lumen of the vessel is even completely occluded. However, because there are abundant collateral vessels around the occluded vessels, clinical symptoms of pulmonary or gastrointestinal ischemia may not be present.
Patients with the thoracoabdominal aorta type may have decreased skin temperature of both lower extremities on physical examination, and the pulsations of the abdominal aorta and bilateral femoral and dorsalis pedis arteries are significantly weakened or absent, and there may be pressure pain in the abdominal aorta and femoral artery. A systolic vascular murmur may be detected in the subxiphoid process at the left edge of the sternum, supraumbilical or interscapular region of the back, and conducts downward. The patient has ischemia in the lower extremities, but there is usually no gangrene at the toe ends. In addition, the patient’s upper extremity blood pressure is significantly elevated, up to 24-32.6/12-18 kPa (180-135 mmHg) or even higher, which cannot be achieved with the usual antihypertensive drugs. The heart also has compensatory enlargement, especially the left ventricular wall is significantly thickened.
(iii) Mixed type
Patients with mixed type have a wide range of vascular involvement and may have the symptoms and signs of the above-mentioned head and arm type, thoracoabdominal aortic type, or renal artery type at the same time. Most of these patients have a limited lesion first and then develop a mixed type later. The renal artery is the most frequently involved; therefore, most patients with the mixed type have significant hypertension, and other clinical manifestations vary according to the different vessels involved. Among the mixed patients, a few patients have also been reported in the literature to have angina pectoris or even myocardial infarction due to coronary artery involvement and aortic valve insufficiency due to dilatation of the aorta after involvement, thus severely affecting cardiac function. There are also reports in the literature about aortitis leading to arterial dilatation and even aneurysm formation.
[Ancillary tests]
1. Laboratory tests: In the early or active stage of the lesion most have increased erythrocyte sedimentation rate and mildly elevated white blood cells. Chronic patients may have mild anemia, decreased plasma albumin, normal platelet count, clotting time and prothrombin time.
2. Chest and abdominal plain film: In patients with thoracoabdominal aortic stenosis, heart enlargement and indentation of the lower border of the rib cage may be found in the chest film. Occasionally, calcification of the aortic wall may be seen.
3. Ultrasonic vascular detection
Different types of ultrasonic angiometry can measure arterial pulsation intensity and blood flow velocity, as well as vascular murmurs or aneurysms. In patients with aortitis, it can be used to determine the pulsation and blood flow of the diseased artery and its distal arteries. This test is simple and does not increase the patient’s pain, and it has some value for understanding vascular lesions and blood flow, so it is widely used in clinical practice.
4.Electrocardiogram: When hypertension lasts for a long time or the lesion involves the aortic valve and coronary artery, the electrocardiogram can show heart enlargement, myocardial damage or myocardial infarction.
5.Fundus examination: If the carotid artery is involved, the cerebral hemogram may show reduced blood flow to the brain.
6.Isotope nephrography and intravenous pyelogram: Patients suspected of having renal artery type of disease can have isotope nephrography and intravenous pyelogram to understand the extent of renal blood flow and renal function changes.
7.Arteriography
Arteriography has certain risks and complications, so it cannot be used as a routine diagnostic method for this disease. When surgical treatment is considered, aortogram is necessary before surgery to understand the location, extent and degree of the lesion in detail. In the early stages of aortitis, the arteriogram shows an irregularly shaped, unsmooth vessel wall with good patency. In the later stages of the disease, lumen thinning, occlusion or occasionally calcification of the aortic wall may be seen. Lesions of the aortic branches are often confined to the beginning of the branch vessels. In and around the diseased artery, abundant collateral vessels are seen. The luminal stenosis has a centripetal character and is generally smooth in outline. Stenosis of the thoracic and abdominal aorta may be wavy in appearance. Stenosis of aortic branches is often more uniform and regular. Occlusion of the lumen is often gradual from narrowing to occlusion. The former is more limited and is seen distal to the stenotic segment, while the latter is often more extensive, but is more or less accompanied by varying degrees of arterial lumen narrowing or occlusion. Sometimes, due to severe stenosis or occlusion of the main artery, the proximal artery adjacent to the lesion site may also dilate due to increased pressure.
[Clinical diagnosis]
The diagnosis of polyarteritis is generally not difficult and relies on a comprehensive analysis of the history, clinical manifestations, and ancillary tests. sharma et al. proposed new diagnostic criteria in 1996, the main indicators of which include: injury to the middle segment of the left subclavian artery, stenosis of the middle segment of the right subclavian artery and/or characteristic signs and symptoms for at least 1 month. Secondary indicators were: elevated sedimentation, carotid artery pressure pain, aortic valve insufficiency, aortic annulus dilatation, pulmonary artery injury, left middle common carotid artery injury, distal head and arm artery trunk injury, thoracic descending aortic injury, abdominal aortic injury, and/or coronary artery injury. A high probability TA diagnosis was obtained if 2 major indicators, or 1 major indicator plus 2 minor indicators, or 4 minor indicators were achieved. Clinically, depending on the type of lesion, specific diagnostic criteria should be mastered.
1.Diagnosis of typical multiple aortitis
(1) Head and arm type: cerebral ischemic symptoms with diminished or absent carotid pulsation, vascular murmur can be heard or upper limb ischemic symptoms with reduced or undetectable blood pressure and diminished or absent radial artery pulsation. Ultrasound examination shows reduced blood flow velocity in the head and arm arteries, and ischemic changes in fundus examination should be considered as head and arm type aortitis.
(2) Thoracoabdominal aortic type: persistent hypertension with reduced or absent arterial pulsation and ischemia in both lower extremities, and vascular murmurs in the thoracoabdominal aorta should be considered as thoracoabdominal aortic type aortitis.
(3) Mixed type: mixed type of aortitis should be considered if the above two combinations of symptoms are present.
2. Diagnosis of atypical polyarteritis
(1) For cases with only low fever, weakness, muscle and joint pain or pressure pain in the region of large vessels should be followed up and observed, because the above-mentioned manifestations may be the early symptoms of aortitis, and pressure pain in large arteries is one of the characteristics of the disease.
(2) Patients with vertigo and transient ischemic attacks should be checked for bilateral carotid pulsations.
(3) In chronic paroxysmal abdominal pain, the possibility of chronic ischemic colic in the intestine due to thoracoabdominal aortic aortitis should be considered after excluding organic lesions of other abdominal organs.
(4) Aortic dilatation due to this disease should be suspected when other causes of aortic valve insufficiency are excluded.
[Differential diagnosis]
Since there is no specific laboratory diagnostic method for this disease, it must be differentiated from other diseases in the diagnostic fashion
1. Connective tissue diseases In the early stage, the disease has non-specific symptoms such as malaise, fever, muscle and joint pains, which are similar to connective tissue diseases, so it should be differentiated from connective tissue diseases such as rheumatoid arthritis, polymyositis, systemic lupus erythematosus, periarteritis nodosa or rheumatic fever when necessary. In order to differentiate from these diseases, necessary laboratory tests should be performed, such as determination of blood rheumatoid factor, anti-nuclear antibody, mucin and anti-“O”, lupus cells, blood uric acid and urine creatine. In case of erythema nodosum, tissue biopsy can be helpful for diagnosis. A small number of patients need to be identified during follow-up. Although some connective tissue diseases may cause occlusion of small limb arteries, no large artery lesions occur.
2, thrombo-occlusive vasculitis The thoraco-abdominal aorta type of aorta can have the manifestation of lower limb artery ischemia, similar to the manifestation of thrombo-occlusive vasculitis, but the latter lesion is mainly located in the middle and small arteries, such as the dorsalis pedis artery, the posterior tibial artery, often with significant resting pain, and later prone to gangrene of the extremity, involving the upper limb vessels less often. Although aortitis can also occur in the lower extremities of the blood supply, but the resting pain is less, and less gangrene.
3, atherosclerotic occlusion This disease occurs mostly in male patients over 40 years of age. The lesions mainly involve the abdominal aorta and the arteries of the lower limbs, and a few cause occlusion of the subclavian artery. The late stage of the disease can cause limb necrosis, often accompanied by hyperlipidemia and diabetes mellitus.
4. Giant cell arteritis, also known as temporal arteritis. The etiology is unknown. In addition to the temporal artery, the lesion may also affect the occipital artery, cerebral artery, carotid artery and brachial artery. It can cause severe cerebral and ocular ischemia due to occlusion of blood vessels. The clinical manifestations are similar to the head and arm type of aortitis. However, the disease mostly occurs in older men, with an average age of onset of about 65 years, and is rare in those under 50 years of age. It is rare in women. The lesion does not involve the thoracoabdominal aorta.
5, thoracic outlet syndrome Due to the abnormal anatomical structure of the thoracic outlet or inflammation and strain of the oblique muscle and local lymph node enlargement, etc., compression of the subclavian artery, vein and brachial plexus nerve can cause the clinical manifestations such as numbness and weakness of the affected upper limb and weakened radial artery pulsation, which is similar to the head and arm type of aortitis. However, patients with thoracic outlet syndrome also have obvious manifestations of brachial plexus nerve compression, such as neck and upper limb venous anger due to subclavian vein compression. On physical examination, fullness of the supraclavicular region with pressure pain is common. The strength of the radial artery pulsation may change with rotation of the head, neck and upper extremities. x-rays sometimes show cervical rib deformities.
[Treatment]
It is important to note that this disease has adverse effects on multiple organs such as the heart, brain and kidney due to lesions in the main vessels. If the lesions are not stabilized for a long time, it will lead to serious consequences, and it is often difficult for any treatment to significantly improve the symptoms at a later stage. For example, in cephalothoracic aortitis, the carotid artery and vertebral artery may be completely occluded in the late stage, leading to blindness and dizziness, and it is difficult to raise the head, even in the head-high and foot-low position, so the patient’s quality of life is very low. At this time, drug treatment is not effective, and surgical diversion is prone to ischemia-reperfusion injury and intracranial hemorrhage. Therefore, early diagnosis of the disease and timely and correct treatment using a combination of Chinese and Western medicine are essential.
Chinese medicine has certain effect, but it should be used according to different types of evidence.
Percutaneous transluminal angioplasty A catheter with a balloon is inserted into the stenosis to perform balloon dilatation and shaping, which is effective for limited stenosis of large vessels and can rapidly improve the symptoms, which is undoubtedly a blessing for such patients. This treatment is easy to perform, with little damage and quick recovery, and can be repeated.
Surgery can be considered for occlusion of the main vessels with good distal outflow tracts. Surgery is usually performed six months to one year after the lesion has stabilized and the clinical examination including temperature, erythrocyte sedimentation rate, and white blood cell count, are normal.
In addition, surgery should be performed when there is no loss of organ function in order to improve blood supply and maintain function.
As the diseased vessels are stiff and atrophied in the later stages, sometimes they become more extensive and have extensive adhesions to the surrounding tissues. Therefore, bypass grafting is generally performed to re-establish blood flow and preserve the established collateral circulation, which is the preferred surgical method for this disease.