OVERVIEW
Chronic non-specific inflammatory disease involving the aorta and its major branches, typically characterized by a weakened or absent pulse, claudication, dizziness, accompanied by fever and arthralgia, etc. The cause of the disease is unclear, and may be related to genetics, autoimmunity, and abnormal levels of estrogen, etc. Treatment with glucocorticosteroids or immunosuppressants is the mainstay of treatment, with surgical procedures being considered when necessary.
Definition
Aortitis is a chronic non-specific inflammatory disease involving the aorta and its major branches. Lesions are most common in the aortic arch and its branches, followed by the descending aorta, abdominal aorta, and renal arteries.
Epidemiology
The disease is prevalent in Asia and the Middle East, with an estimated incidence of 15/100,000 in Japan and (0.2-2.6)/1 million in Europe and the United States.
The disease is more common in young women before the age of 30 and less common after the age of 40, with a male-to-female prevalence ratio of 1: (8-9).
Typing
Currently widely recognized is the vascular typing of aortitis proposed by Numano. According to the different vessels involved, aortitis can be categorized into 5 types.
Cephaloarterial type: stenosis and occlusion of the carotid, subclavian, and vertebral arteries.
Thoracic-abdominal aortic type: involvement of the aorta, abdominal aorta and/or branches.
Renal artery type: involvement of the descending aorta and bilateral renal arteries.
Pulmonary artery type: involves unilateral or bilateral pulmonary lobar or segmental arteries, often in combination with other types.
Extensive: involves the entire length of the aorta and its first-degree branches.
Causes
Causes
The cause of the disease is unknown. The following factors may be associated with the disease.
Genetic factors
The disease is associated with certain dominant genetic factors.
Autoimmune factors
It is hypothesized that after infection with microorganisms (e.g., Mycobacterium tuberculosis, viruses, etc.), antigens within the walls of the aorta are exposed, antibodies against the walls of the aorta are produced, immune complexes are formed and deposited in the walls of the aorta, and nonspecific inflammation occurs.
Abnormal estrogen levels
This disease is most common in young women before the age of 30, and high estrogen levels may be associated with the development of this disease.
Pathogenesis
The pathogenesis of aortitis is complex and still unclear. Vascular injury in aortitis mainly involves cellular immunity, with T-cell-dependent immune responses, chemokine-cytokine-dependent immune responses, and, controversially, B-cell-dependent immune responses, being the main mechanisms leading to vascular injury.
Lesioned blood vessels are grayish-white, with stiff, calcified, and atrophied walls, adhesions to surrounding tissues, and narrowed or occluded lumens.
Symptoms
Main Symptoms
The clinical manifestations of the five types of aortitis vary due to the different vessels involved.
Head and arm artery type
Involvement of carotid artery and vertebral artery mainly manifests as dizziness, headache, insomnia, memory loss, vision loss, etc. In severe cases, blindness, aphasia, fainting, hemiparesis, convulsions, coma, and even life-threatening.
Involvement of subclavian artery or axillary artery mainly manifests as weakness, coldness, soreness and numbness of the upper limbs, etc. Physical examination may reveal that the radial artery pulsation is weakened or disappeared, and pulselessness may occur.
Thoracic-abdominal aortic type
Dilatation of the aortic root can lead to aortic regurgitation, which can lead to heart failure.
In some patients, coronary artery involvement may result in angina pectoris.
Branches of the abdominal aorta and lower limb arteries may also be involved in a few patients, resulting in abdominal pain and intermittent claudication of the lower limbs.
Chest and back pain may occur in some patients.
Renal artery type
Renal artery stenosis is mainly characterized by intractable hypertension, especially elevated diastolic blood pressure, which may be manifested as dizziness and panic.
Pulmonary artery stenosis
This type is relatively rare, mainly manifested as cough, chest pain, shortness of breath or hemoptysis.
Extensive type
This type may present all the above clinical manifestations, and most patients are in more serious condition.
Systemic symptoms
Inflammation is the main manifestation, mainly including fever, general malaise, night sweats, arthralgia, anorexia, weight loss, and occasionally oral ulcers and erythema nodosum, etc. The disease may have acute onset or insidious onset.
As the disease progresses, the systemic symptoms may gradually decrease or disappear, and some patients may not have systemic symptoms.
Complications
Hypertension
Mainly due to renal artery involvement, headache, dizziness, palpitations and other manifestations may be present.
Pulmonary hypertension
The most common first symptom is shortness of breath and fatigue after activity.
Other symptoms include chest pain, hemoptysis, vertigo, syncope, and dry cough.
Cerebrovascular disease
Carotid artery and vertebral artery stenosis and occlusion cause different degrees of ischemia in the brain.
There may be headache, dizziness, blurred consciousness, abnormal sensation and other symptoms.
Heart failure
Involving the pulmonary artery and aortic valve leads to.
It may be characterized by weakness, dizziness, cough, sputum, hemoptysis, palpitation and dyspnea.
Kidney failure
Renal insufficiency due to insufficient blood supply to the kidneys, and in severe cases, renal failure may occur.
Multi-system symptoms such as low or no urine, generalized edema, nausea and vomiting may occur.
Myocardial infarction
Caused by the involvement of coronary artery, mainly manifested as sudden persistent pressure-like pain behind the sternum, accompanied by a sense of near death.
Consultation
Conditions that require medical attention
Women under 40 years of age who develop elevated blood pressure, weakened pulse in both arms, pulselessness in the limbs, or prolonged unexplained fever; or who develop headache, dizziness, blurred vision, loss of visual acuity, weakness in chewing, neck pain, weakness in the limbs, and intermittent claudication need to consult a doctor promptly.
If you have difficulty breathing, palpitations, fainting, hemoptysis, etc., someone close to you should immediately send the patient to the emergency department, or call “120” to call an ambulance.
Suggested departments
Rheumatology, Cardiovascular Medicine, Vascular Surgery
Preparation
If there are no special circumstances, you need to register through the hospital’s official website, official app, 114 and other regular channels, prepare the social security card (medical insurance card) and other medical information, bring the previous medical information, and apply for the record for patients who seek medical treatment in other places.
Emergency department visits can be registered directly, without the need to register through the above channels. The social security card and previous medical documents can be replenished at any time during the visit. There is no need to register for medical treatment in a different location.
What questions the doctor may ask
What are the symptoms?
How long have these symptoms been present?
Under what circumstances do the symptoms worsen or lessen?
Is there a history of other systemic diseases?
Has anyone in the family had aortitis or other autoimmune disease?
Have you had any relevant tests? What were the results?
Have you had any treatment? What was the treatment? What is the effect?
Any history of drug allergy, surgery, abortion, etc.?
Are you taking any medication? If so, which ones?
Diagnosis
Disease Diagnosis
Diagnostic basis
The diagnosis is made after a comprehensive analysis of the medical history, clinical manifestations, and examination findings, and after other diseases have been ruled out.
Medical history
History of infection, autoimmune disease or other systemic diseases.
Clinical manifestations
Symptoms
Presentation of headache, dizziness, blurred vision, decreased visual acuity, weakness in chewing, neck pain, weakness of limbs, intermittent claudication.
With or without systemic symptoms such as fever, general malaise, night sweats, arthralgia, anorexia, weight loss, oral ulcers and erythema nodosum.
Physical Examination
Blood pressure: reduced or undetectable blood pressure; bilateral blood pressure asymmetry; recent hypertension or persistent hypertension.
Arterial pulsation: unilateral or bilateral arterial pulsation is weakened or absent.
Vascular murmurs: vascular murmurs can be heard in the carotid artery, subclavian artery auscultation area, abdominal or renal artery auscultation area.
Laboratory tests
Including routine blood tests, erythrocyte sedimentation rate, C-reactive protein, autoantibody tests.
Routine blood tests: the white blood cell and platelet counts may be elevated in the acute phase or during the active phase of the disease.
Increased erythrocyte sedimentation rate and elevated C-reactive protein can be seen in the active phase.
Autoantibody test: some patients may have positive anti-endothelial cell antibody and anti-aortic antibody.
Imaging
Color Doppler ultrasound: It can detect stenosis or occlusion of the aorta and its major branches (carotid, subclavian, renal, etc.), and in a few cases, vasodilatation or hemangioma may be seen. Ultrasound is more difficult to detect the distal branches of the vessels.
CT angiography (CTA)
It can indicate the narrowing and dilatation of the lumen of the main renal artery and its primary and secondary branches.
Calcification, entrapment, plaque and hemorrhage are seen in the walls of the renal arteries.
The anatomy of the abdominal aorta and branches and the presence of pararenal arteries.
Digital Subtraction Angiography (DSA): Helps to detect arterial stenosis, occlusion, intracranial aneurysms and arteriovenous malformations. However, it does not show well for small arteries within the organs.
Magnetic Resonance Imaging (MRI): suggests edema in the walls of the affected vessels and helps determine the activity of the disease.
Positron emission tomography (PET): helps to observe the uptake of isotopes by the vessel wall, and can be used to determine the activity and degree of disease.
Echocardiography: It can show the stenosis and dilatation of the thoracic and abdominal aorta, as well as the stenosis of the pulmonary artery and the proximal segment of the aortic branches.
Funduscopic examination
Helps to determine the presence of fundus lesions caused by ischemia of the ophthalmic arteries.
Diagnostic Criteria
The 1990 American College of Rheumatology (ACR) classification is generally used. The disease is diagnosed if three of the following six criteria are met.
Age of onset ≤40 years.
Intermittent claudication of the limbs, with progressively increasing weakness and muscle discomfort in one or more limbs during activity, especially in the upper limbs.
Weakness of brachial artery pulsation on one or both sides.
Difference in systolic blood pressure between upper limbs >10 mmHg.
A vascular murmur is heard in one or both subclavian arteries or abdominal aorta.
Angiographic abnormalities, stenosis or occlusion of first-degree branches of the aorta or large arteries proximal to the upper and lower extremities; lesions are often focal or segmental and are not due to atherosclerosis, fibromuscular dysplasia, or similar causes.
Differential diagnosis
Fibromuscular dysplasia
Similarity: Fibromuscular dysplasia is more common in young women, often involving the renal artery and internal carotid artery, and can be characterized by high blood pressure, palpitations, fatigue and dizziness.
Differences: Fibromuscular dysplasia is a non-inflammatory, non-atherosclerotic arterial vascular disease. Typical angiographic manifestations are “bead-like changes”. Combined with ultrasound, CTA, angiography, etc., the diagnosis can be clarified.
Atherosclerosis
Similarity: no specific symptoms in the early stage, then dizziness, chest tightness and other manifestations may appear.
Difference: Atherosclerosis is not difficult to diagnose when it has developed to a considerable degree, especially when there are obvious lesions in organs. Elderly people with dyslipidemia and arteriographic findings of stenotic lesions can help in differential diagnosis.
Behçet’s syndrome
Similarities: Both may present with symptoms of heart, lung and blood vessel damage.
Differences: Behçet’s syndrome is a chronic systemic vascular inflammatory disease clinically characterized by oral and vulvar ulcers, ophthalmia, and may involve multiple systems such as intestines and nerves. Typical clinical manifestations and pinprick reaction tests are helpful in the differential diagnosis.
Polyarteritis nodosa
Similarities: Both may present with symptoms of heart and kidney damage.
Differences: Polyarteritis nodosa is common in men aged 50-60 years old, and mostly involves medium-sized arteries of the limbs, gastrointestinal tract, liver and kidneys, while the lungs and glomeruli are not involved.
Thromboembolic vasculitis
Similarity: symptoms such as ischemia, pain, intermittent claudication, and weakening or disappearance of pulsation of the affected arteries.
Differences: Thromboembolic vasculitis mostly involves the middle and small arteries and veins of the limbs, with the lower limbs being the most common, and it is more common in male young adults.
IgG4-related aortitis
Similarity: Both may present with inflammation and stenosis of the aorta and periaorta.
Differences: IgG4-associated aortitis has elevated serum IgG4 levels and abundant IgG4-positive plasma cells and matted fibrotic changes on pathologic examination.
Treatment
Aim of treatment: to control active lesions and relieve organ ischemia.
Treatment methods: including general treatment, drug treatment, surgical treatment and so on.
General treatment
Correctly recognize and view the condition, and communicate with family members to maintain a good psychological state and establish optimism.
Pay attention to the changes of your condition and go to the hospital in time once the symptoms worsen or recur.
Medication
During drug treatment, you should adhere to the doctor’s instructions, do not change the dose of drugs without authorization or suddenly stop the drug, to ensure that the treatment plan is implemented. You should know the name, dosage, time and method of medication.
Glucocorticoid
Glucocorticosteroids are the basic drugs for the treatment of aortitis.
Prednisone and methylprednisolone can be used.
Active severe cases can be tried high-dose methylprednisolone intravenous shock treatment.
If the clinical symptoms are relieved and stabilized, the dosage can be reduced and discontinued as prescribed by the doctor.
Long-term application of glucocorticoids should pay attention to infection, osteoporosis, gastrointestinal bleeding, hypokalemia and other adverse reactions.
Immunosuppressant
Immunosuppressants combined with glucocorticoids can enhance the therapeutic effect.
Cyclophosphamide, methotrexate and azathioprine are commonly used.
In the treatment, attention should be paid to checking blood and urine routine and liver function and renal function to monitor the occurrence of adverse reactions.
Biological agents
In recent years, it has been reported that tumor necrosis factor (TNF)-α inhibitors and interleukin 6 (IL-6) inhibitors can improve the symptoms and inflammatory indexes of patients with aortitis, but further clinical studies are needed to confirm this.
Other drugs
Antihypertensive drugs: commonly used drugs include calcium antagonists, angiotensin-converting enzyme inhibitors, angiotensin receptor antagonists, diuretics β-receptor antagonists and so on.
Surgery
Most vessels have abundant collateral circulation, therefore premature vascular intervention is not recommended and surgery should not be performed during active disease if possible.
The aim of surgical treatment is mainly to perform hemodialysis and to treat severe stenosis or aneurysm of the vessel that is not responding to pharmacological treatment.
Bypass surgery for stenosis or aneurysms, aortic valve replacement for severe aortic regurgitation, and percutaneous transluminal angioplasty for renal artery stenosis resulting in severe hypertension may be performed.
Prognosis
Cure
Aortitis is self-limiting in 20% of patients, while the remaining patients have chronic lesions that require long-term pharmacologic therapy.
The affected arteries may form collateral circulation, so the prognosis is good if the blood supply to vital organs is not involved.
Hazards
Important complications, such as retinopathy, hypertension, assisted arterial reflux and hemangiomas, or progressive disease, can seriously affect normal work and life.
Combined with heart failure, renal failure, cerebrovascular events or aneurysm rupture, the prognosis is poorer, and may even be life-threatening.
If long-term use of glucocorticoids prone to infection, osteoporosis, hypertension, hyperglycemia, hyperlipidemia and other adverse effects, may affect the health and quality of life.
Daily life
Daily life
Diet
Eat more fresh vegetables, fruits, lean meat and other high-protein, high-vitamin and easy-to-digest foods.
Avoid spicy and stimulating foods such as alcohol, strong tea, coffee, raw garlic, ginger, chili peppers and curry.
Avoid high sugar diet, such as drinks, candies, snacks, etc., to prevent blood sugar fluctuation and inflammation exacerbation.
If renal insufficiency, choose low-salt, high-quality low-protein diet and follow the doctor’s advice to limit the amount of water consumption.
Combined hypertension, hyperlipidemia, cardiac insufficiency, etc., should avoid eating fried and pan-fried food.
Living Habits
Pay attention to a neat and clean environment, and open the windows regularly for ventilation every day.
During the period of disease remission, you can do some gentle exercise, such as walking, tai chi, radio gymnastics, etc., which can help to enhance physical fitness, improve immunity and avoid muscle atrophy.
Do not watch TV or use computer for a long time to avoid increasing eye fatigue.
Pay attention to eye hygiene and avoid rubbing your eyes with your hands.
Avoid going to places where people gather.
If there is numbness and coldness in the limbs, strengthen protection, pay attention to keep warm and avoid trauma.
Strictly quit smoking and avoid exposure to “second-hand smoke”.
Psychological care
Face the disease with a positive and optimistic mindset, and establish confidence in overcoming the disease.
Talk to your family, partner and friends about your feelings.
Strive to take care of your own life or participate in jobs that you can do to enrich your life.
If severe anxiety, depression, insomnia, etc. occur, it is recommended to go to the hospital for regular psychological treatment.
Regular follow-up
Follow the doctor’s prescribed time for follow-up consultation.
Seek timely medical treatment for any changes in your condition
If symptoms worsen or new symptoms appear, consult a doctor promptly.
Prevention
Actively treat autoimmune or other systemic diseases.
Eat a balanced diet with a variety of foods.
Avoid enclosed public places with poor air circulation and crowded places during the epidemic season.
Ensure good mood and adequate sleep, and keep a regular schedule.