Takayasu arteritis is known as the "Oriental beauty disease". Is there any way to prevent and treat it?

Author: Pan Lili, Chief Physician, Beijing Anzhen Hospital, Capital Medical University

Reviewer: Mu Rong, Chief Physician, Peking University Third Hospital

Vice Chairman of the Young and Middle-aged Committee of the Chinese Medical Association Rheumatology Branch

Takayasu arteritis is an autoimmune disease, a type of vasculitis, that primarily affects the largest artery in the human body, the aorta and its primary branches.

Figure 1 Original copyright image, no permission to reprint

Takayasu arteritis is common in young Asian women under the age of 40, so it is called "Oriental beauty disease."

1. What is the cause of Takayasu arteritis?

As with other autoimmune diseases, the cause of Takayasu arteritis is unclear.

It has been found that human leukocyte antigen HLA-B52 is closely related to Takayasu arteritis. It is a susceptibility gene, which is also a possible reason for its high incidence in young Asian women.

In addition, Takayasu arteritis may also have a certain relationship with estrogen, but there is no conclusion yet. It is just a speculation, and it is also speculated based on the characteristics of the current disease population.

For example, infection by pathogenic microorganisms may serve as a predisposing factor for Takayasu arteritis.

Takayasu arteritis is a disease of unknown cause. Since the cause is unknown, there is no way to take targeted prevention measures.

2. Are there any early symptoms of Takayasu arteritis?

Takayasu arteritis is caused by chronic inflammation of the vessel wall, which leads to uniform thickening of the vessel wall and gradual narrowing and occlusion of the lumen. This process often takes several years or even decades, so most patients do not have any clinical symptoms in the early stages and are difficult to detect.

Patients with more severe arterial inflammation may experience systemic manifestations, such as fever, fatigue, weight loss, localized vascular pain, such as neck pain, etc. The clinical manifestations of large arteritis are mainly caused by vascular stenosis or occlusion. Severe stenosis or occlusion of important blood vessels causes ischemia of important organs. It will not be discovered or noticed until the corresponding ischemic symptoms appear.

For example, occlusion of the neck blood vessels can cause cerebral infarction; occlusion of the subclavian artery can cause pulselessness; stenosis or occlusion of the renal artery can cause persistent hypertension; stenosis or occlusion of the iliac artery can cause intermittent claudication; stenosis or occlusion of the coronary artery can lead to angina pectoris or myocardial infarction.

It is difficult to detect Takayasu arteritis in its early stages. Sometimes it can be discovered unintentionally, such as during a physical examination. Once discovered, it must be actively treated to avoid involvement of important organs.

3. How is Takayasu arteritis diagnosed?

Currently, according to the diagnostic criteria of the American College of Rheumatology in 1990, there are six items. If three of them are met, it can basically be diagnosed as Takayasu arteritis.

First, the age of onset is ≤40 years old;

Second, the pulse weakens or disappears;

Third, there is limb movement disorder. Blood vessel stenosis and occlusion cause limb ischemia, which will cause progressive fatigue and pain during activities;

Fourth, the systolic pressure of both arms is not equal, and the pulse pressure difference exceeds 10mmHg. If the starting part of the left subclavian artery is blocked, the pulse on the left side will weaken and disappear, and the blood pressure on the left upper limb will decrease. If there is no lesion on the right side and the blood pressure is normal, the pulse pressure difference on both sides will exceed 10mmHg.

Fifth, vascular murmurs can be heard during auscultation of the aorta and its branches;

Article 6. Imaging examination shows signs specific to Takayasu arteritis.

In 2018, the American College of Rheumatology and the European League Against Rheumatism jointly launched a new classification standard, which expanded the previous standard and relaxed the age limit to ≤60 years old. A points system was implemented according to the site of vascular involvement: 1 point for women; 2 points for angina pectoris caused by vasculitis; 2 points for upper and/or lower limb claudication; 2 points for arterial murmur; 2 points for weakened upper limb artery pulsation; 2 points for weakened or tender carotid artery pulsation; 1 point for blood pressure ≥20 mmHg in both upper limbs. In terms of imaging, 1 point is scored if one vascular site is affected, 2 points if two vascular sites are affected, 3 points if three vascular sites are affected, and an additional 1 point is scored if paired vascular sites are affected; 3 points are scored if abdominal vascular involvement accumulates. A score of 5 or more can be diagnosed with Takayasu arteritis.

Both criteria can be used to diagnose Takayasu arteritis, but the latter is relatively more complicated, so the 1990 standard is still used as the standard.

4. How to treat Takayasu arteritis?

It mainly includes symptomatic treatment, medical treatment and surgical treatment, with internal medicine treatment as the main focus.

For symptomatic treatment, such as the use of antihypertensive drugs for refractory hypertension in patients with renal artery stenosis.

The most commonly used drugs for the medical treatment of Takayasu arteritis are glucocorticoids plus immunosuppressants. The latest European League Against Rheumatism Takayasu arteritis treatment guidelines in 2019 mentioned that if the disease is active, it is necessary to use relatively large doses of glucocorticoids in the early stage of treatment. Only in this way can the inflammation be quickly and effectively controlled, and then the dose can be gradually reduced according to the disease control situation.

During the process of reducing the dosage of hormones, it is easy to relapse, so immunosuppressants need to be used in combination at this time. First, they can control the inflammatory response of large artery inflammation, and second, they can help reduce the dosage of hormones.

If the blood vessels of important organs are affected, such as the coronary arteries or renal arteries, and the blood vessels become narrowed or occluded, posing a life-threatening situation, a surgeon will be needed to help determine whether to perform appropriate surgical treatment, such as stent placement or bypass surgery.

According to the severity and progression of the disease, regular evaluation should be carried out and the drug treatment plan should be adjusted in a timely manner.

Takayasu arteritis is a chronic disease that is currently incurable. If it is detected in the early stages, it can be controlled through active and regular treatment and regular follow-up, and you can live with the disease without affecting your normal life.