Takayasu arteritis
OVERVIEW
What is a large artery?
As the name suggests, a large artery refers to an artery with a large diameter. Because its wall is rich in elastic fibers and contains less smooth muscle, it is also called an elastic artery. Commonly seen examples include the aorta, brachiocephalic trunk, subclavian artery, common carotid artery, pulmonary artery, etc., all of which fall under the category of large arteries.
What is Takayasu arteritis?
This is a chronic inflammation that occurs in large arteries. Symptoms may include general discomfort, fatigue, fever, loss of appetite, excessive sweating, weight loss, as well as various symptoms caused by tissue or organ ischemia due to vascular stenosis or occlusion.
Is Takayasu arteritis a common disease?
It is not a common disease, and it can sometimes be missed due to the absence of obvious symptoms.
SYMPTOMS
What are the types of Takayasu arteritis?
Based on the affected arteries, Takayasu arteritis can be divided into 5 types:
- Brachiocephalic type: Lesions occur in the major branches of the aortic arch, such as the common carotid artery, subclavian artery, and innominate artery.
- Thoracoabdominal aortic type: Primarily affects the thoracic aorta and/or abdominal aorta, often leading to stenosis, occlusion, or aneurysmal dilation.
- Renal artery type: Involves stenosis or occlusion of the renal artery, sometimes extending to intrarenal arteries, causing renal ischemic hypertension, renal failure, and related symptoms.
- Mixed type: Features two or more of the above types, with broader vascular involvement, most commonly including the renal artery.
- Pulmonary artery type: Affects the main, lobar, or segmental pulmonary arteries, leading to diffuse or segmental stenosis, most frequently in the right upper lobe and left lower lobe arteries.
What are the symptoms of Takayasu arteritis?
During onset, systemic symptoms may include fever, fatigue, poor appetite, weight loss, excessive sweating, and menstrual irregularities. In mild or inactive cases, symptoms may be absent. Some patients present atypically, such as with unexplained fever or pericardial effusion.
Additionally, symptoms vary by affected arteries, involving vascular, neurological, cardiac, or pulmonary manifestations.
Patients should monitor blood pressure and cerebral blood flow. Complications like hypertension or cerebral ischemia may lead to cerebral hemorrhage, thrombosis, heart failure, renal failure, myocardial infarction, aortic valve regurgitation, or blindness.
Can Takayasu arteritis be fatal?
Severe cases may be life-threatening. Common causes of death include cerebral hemorrhage, renal failure, heart failure, aneurysm rupture, and pulmonary embolism.
CAUSES
What causes Takayasu arteritis?
Takayasu arteritis differs from the typical "inflammation" we commonly refer to, as it does not involve a clear pathogenic bacterial infection.
The exact cause of Takayasu arteritis remains incompletely understood. Most perspectives suggest it is an autoimmune disease, where the body's immune response targets its own antigens (i.e., tissues), leading to tissue damage. Additionally, Takayasu arteritis may also be associated with genetic factors, endocrine abnormalities, and other influences.
Who is most susceptible to Takayasu arteritis?
This disease predominantly affects young women, with a male-to-female ratio of approximately 1:3. Most patients develop symptoms between the ages of 12 and 30.
Is Takayasu arteritis hereditary?
Current research suggests that Takayasu arteritis has a certain genetic probability and a tendency for familial occurrence.
What factors can worsen Takayasu arteritis?
Since Takayasu arteritis may be related to genetic factors, endocrine abnormalities, immune dysfunction after infection, and inflammatory cytokine responses, the following conditions can exacerbate the disease:
- Unhealthy lifestyle habits (e.g., lack of exercise, smoking, excessive alcohol consumption, high-salt or high-fat diet);
- Infections;
- Poorly controlled hypertension;
- Progression or worsening of underlying conditions such as autoimmune diseases.
DIAGNOSIS
What tests are needed for Takayasu arteritis?
- Physical examination: Includes palpating arterial pulses throughout the body, auscultating for arterial bruits, measuring blood pressure in the limbs, etc.
- Blood tests: Including complete blood count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), coagulation function, immune markers, aortic antibodies, anti-streptolysin O (ASO), rheumatoid factor, etc.
- Ultrasound examination: Vascular ultrasound can be used to diagnose Takayasu arteritis and assess the degree of arterial stenosis and occlusion; ocular ultrasound has some diagnostic value for severe stenosis or occlusion of the internal carotid artery. Ultrasound is painless and radiation-free, making it widely applicable.
- Angiography (DSA): Angiography is the primary basis for diagnosing Takayasu arteritis and is more precise than ultrasound.
- Others: Depending on the condition, selective tests such as X-rays, CT, MRI, intravascular ultrasound imaging, and radionuclide angiography may be performed.
What is angiography (DSA)?
Angiography is an interventional diagnostic method where a contrast agent is injected into blood vessels and then visualized under X-ray. Angiography can accurately reflect the location and extent of vascular lesions.
What are the diagnostic criteria for Takayasu arteritis?
The currently widely accepted diagnostic criteria are as follows. Meeting at least 3 of the following 6 criteria can diagnose Takayasu arteritis:
- Age of onset under 40;
- Muscle fatigue or worsening fatigue in one or more limbs, especially the arms;
- Weakened arterial pulses in the upper limbs;
- A systolic blood pressure difference of more than 10 mmHg between the two arms;
- Bruits heard over one or both subclavian arteries or the abdominal aorta;
- Abnormal angiography findings.
What diseases should Takayasu arteritis be differentiated from?
Due to similarities in some clinical manifestations, Takayasu arteritis must be distinguished from the following diseases:
- Atherosclerotic diseases;
- Thoracic outlet syndrome;
- Polyarteritis nodosa;
- Fibromuscular dysplasia of the renal artery;
- Congenital aortic coarctation;
- Thromboangiitis obliterans, etc.
Is Takayasu arteritis the same as vasculitis?
No.
Vasculitis is short for thromboangiitis obliterans, commonly presenting with intermittent claudication of the lower limbs and predominantly affecting young male smokers.
It typically involves small and medium-sized arteries and veins in the limbs, may cause migratory phlebitis (a recurring inflammation along superficial veins characterized by sudden redness, heat, pain, or tenderness with cord-like or nodular lesions), and often leads to limb gangrene. It is a different disease from Takayasu arteritis.
TREATMENT
Which department should patients with Takayasu arteritis visit?
Vascular surgery or rheumatology and immunology department.
How is Takayasu arteritis treated?
Treatment for Takayasu arteritis includes surgical and non-surgical (conservative) approaches.
The principle of treatment is to restore blood flow to distal arteries as much as possible, improve blood supply to organs or limbs, reduce ischemia or necrosis, and ensure the patient's quality of life.
Which patients with Takayasu arteritis should receive conservative treatment?
Patients in the active or early stages of Takayasu arteritis are advised to undergo conservative treatment. Clinically, the erythrocyte sedimentation rate (ESR) is primarily used to assess disease activity. If the ESR remains abnormal, conservative treatment should be prioritized.
What are the conservative treatments for Takayasu arteritis?
- For early-stage or active-phase patients, glucocorticoids (e.g., prednisone, dexamethasone) can be administered orally to counteract inflammation. If hormone therapy is ineffective, immunosuppressants (e.g., azathioprine) may be considered.
- Anti-platelet drugs (e.g., aspirin) can be used to prevent thrombosis.
- Patients with cerebral or limb ischemia may benefit from vasodilators (e.g., hydralazine, captopril) to improve circulation.
- Patients with refractory hypertension should receive antihypertensive drugs to prevent severe complications.
- In cases of infection, antibiotics should be administered to control the infection and halt disease progression.
The above treatments should be tailored to the patient's condition under the guidance of a specialist.
When is surgical treatment considered for Takayasu arteritis?
Surgery is typically performed after the condition stabilizes, with normalized body temperature, ESR, white blood cell count, and IgG levels. The goal is to perform arterial reconstruction before organ function is lost, restoring blood supply and preventing ischemic necrosis.
What surgical options are available for Takayasu arteritis?
- For brachiocephalic artery involvement, artificial vascular reconstruction (via thoracic or extra-thoracic approaches) or thrombectomy may be performed. Severe stenosis of the thoracic or abdominal aorta may require artificial vascular reconstruction.
- For renal artery stenosis, kidney autotransplantation or vascular reconstruction may be considered. Nephrectomy is an option if the affected kidney has significantly atrophied.
- Coronary artery stenosis may be treated with coronary artery bypass grafting or stent placement.
What are the risks of surgery for Takayasu arteritis?
Surgical treatment typically targets arteries supplying vital organs, with risks varying based on the organ involved, disease severity, and surgical approach.
Minimally invasive interventions generally carry lower risks due to reduced trauma, whereas open surgeries involve higher risks, including anesthesia-related complications. Patients considering surgery should consult a vascular surgeon for personalized advice.
Is minimally invasive treatment an option for Takayasu arteritis?
In vascular surgery, "minimally invasive" treatment often refers to endovascular or interventional therapy, involving percutaneous placement of guidewires, catheters, balloons, or stents. Due to its low trauma and comparable efficacy to open surgery, it has become a preferred option for vascular occlusive diseases.
For Takayasu arteritis causing severe arterial stenosis and organ ischemia, minimally invasive treatment can be prioritized and often yields satisfactory outcomes.
Can Takayasu arteritis be cured?
Takayasu arteritis is a chronic, progressive disease with alternating remission and relapse phases, making a complete cure unlikely.
Current treatments aim to alleviate symptoms, preserve limb or organ function, and maintain quality of life. Many patients transition to remission naturally or with treatment.
Can medications for Takayasu arteritis be discontinued?
Medications for Takayasu arteritis include glucocorticoids, immunosuppressants, antiplatelet drugs, vasodilators, and antihypertensives. After remission and ESR normalization, glucocorticoids and immunosuppressants may be tapered or discontinued under medical supervision. Other medications should be adjusted based on disease progression.
Patients should never self-adjust or stop medications without consulting a doctor, as this may worsen or reactivate the disease.
DIET & LIFESTYLE
What are the key considerations for postoperative rehabilitation of Takayasu arteritis?
- First, strictly follow medical advice and take long-term medications such as Plavix and aspirin;
- Second, avoid infections and control underlying conditions like hypertension, which are triggering factors for Takayasu arteritis;
- Third, maintain moderate exercise, quit smoking and alcohol, avoid high-salt and high-fat diets, and adopt a healthy lifestyle.
PREVENTION
Can Takayasu Arteritis Be Prevented?
The exact cause of Takayasu arteritis is not yet fully understood, so there is no definitive prevention method.