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Did you know how Takayasu's arteritis makes people feel pulseless in their hands and feet?
Takayasu's arteritis (TA), also known as aortic arch syndrome and pulseless disease, is a granulomatous vasculitis of large blood vessels that mainly affects young or middle-aged women, especially Asian women. , although anyone can be affected. This lesion most commonly affects the aorta and its branches and may also affect the pulmonary arteries.
According to research, women are approximately 8 to 9 times more affected than men.
Many patients develop symptoms between the ages of 15 and 30. In most Western countries, atherosclerosis is the leading cause of aortic arch vessel occlusion, whereas Takayasu arteritis is relatively uncommon.
Because this condition causes blockage of major branches of the aorta, such as the left carotid, brachial and left subclavian arteries, patients often experience weak or absent pulses in the arms, hands, and wrists. This is why this disease is often called "pulseless disease."
Symptoms of disease
People often experience an initial "inflammatory phase" characterized by systemic symptoms including malaise, fever, night sweats, weight loss, joint pain and fatigue, and even syncope. Syncope may be caused by subclavian steal syndrome or carotid sinus hypersensitivity.
In this early stage, anemia and markedly elevated levels of the erythrocyte sedimentation rate (ESR) or C-reactive protein are common on blood tests.
Thereafter, the patient may enter a second phase, the "pulseless phase," which is characterized by insufficient vascular oxygen supply due to narrowing of the arterial lumen, manifested by intermittent claudication of the extremities, renal artery High blood pressure caused by stenosis, and neurological symptoms caused by reduced blood flow to the brain.
Effects of renal artery stenosis
The effects of renal artery stenosis can lead to the development of hypertension. The kidneys with normal oxygen supply will produce a certain amount of renin, but stenosis of the renal arteries will lead to reduced blood flow near the renal tubules, which will lead to increased secretion of renin, eventually leading to water and salt retention and hypertension.
Nervous system symptoms vary depending on the degree and nature of the blood vessel obstruction and can range from dizziness to epilepsy (in severe cases).
In addition to the symptoms mentioned above, Takayasu arteritis may also present with eye symptoms, such as visual field defects, vision loss, or retinal hemorrhage. Some individuals may even present with only advanced vascular changes, without prior systemic disease. In the later stages of the disease, the artery walls may weaken, leading to localized aneurysms, which require regular monitoring to prevent rupture and bleeding.
Causes and diagnosis
Although the specific cause of Takayasu's arteritis is unknown, the disease is characterized by localized granulomatous inflammation of the aorta and major branches, leading to arterial stenosis and thrombosis. Examination of arterial narrowing and irregularities using magnetic resonance angiography (MRA), computed tomography angiography (CTA), or angiography (DSA) can provide the basis for diagnosis.
FDG PET scans are excellent at detecting active inflammation and can help diagnose patients receiving immunosuppressive therapy.
Characteristic "skip lesions" are evident on angiography, meaning the appearance of stenoses or aneurysms alternates between normal vessels.
Treatment
Most people with Takayasu's arteritis respond well to steroids (such as acetaminophen). The starting dose is approximately 1 mg per kilogram of body weight (approximately 60 mg daily for most people). The dosage used is gradually reduced over time to avoid the side effects of long-term high-dose steroid use.
If the patient does not respond to steroid treatment, revascularization surgery may be required.
While current treatments can control symptoms, long-term damage or even death is possible if the standard of care is not high enough. In recent years, research into this disease has provided hope, such as the application of mycophenolate and tocilizumab, which have produced good results for patients.
Medical history
The first case of Takayasu's arteritis was described in 1908 by Japanese ophthalmologist Mikito Takayasu. Takayasu noted at the time a peculiar "garland-like" appearance of blood vessels, and two other doctors reported similar eye findings.
It is now known that these vascular changes are caused by narrowing of the arteries in the neck and are associated with a failure to feel a pulse. The typical symptoms of this disease are relatively rare in patients in North America and British Columbia, but its scope is significant compared to other granulomatous diseases.
By understanding Takayasu's arteritis and the pulseless phenomenon it causes, can we gain a deeper understanding of other similar diseases?
