Takayasu Arteritis
Complete information on the causes, symptoms, and treatments of inflammation of the aorta and its branches.
Symptoms and Causes
Takayasu arteritis is a type of vasculitis, that is, an uncommon inflammation of blood vessels, primarily affecting medium- or large-caliber arteries, especially the aorta and its main branches (supra-aortic trunks).
In some cases, Takayasu vasculitis occurs in a single phase, but in most instances, it is a chronic disease characterized by alternating periods of remission and relapse over time.
Depending on the location of the lesions, Takayasu arteritis is classified into the following types:
- Type I: The branches of the aortic arch are affected, which is the curved segment of the aorta located in the upper thorax, including the brachiocephalic trunk, left common carotid artery, and left subclavian artery.
- Type IIa: Damage occurs in the ascending aorta as well as the aortic arch and its branches.
- Type IIb: Lesions involve the ascending aorta, descending aorta, and the aortic arch with its branches.
- Type III: Lesions occur in the descending aorta and the renal arteries, and sometimes also in the abdominal aorta.
- Type IV: The renal arteries are affected, and in some cases, the abdominal aorta is also involved.
- Type V: Simultaneous involvement of the areas affected in types IIb and IV.
Takayasu arteritis is a serious condition with a variable prognosis, largely dependent on the complications it causes and the timing of treatment initiation, as early detection significantly improves outcomes. In recent years, patient life expectancy has increased considerably, with five-year survival estimated at 95% if adequate treatment is administered.
Symptoms
The symptoms of Takayasu arteritis vary among patients and typically appear progressively in two consecutive phases. The most prominent are:
- Phase 1:
- Fatigue
- Unexplained weight loss
- Muscle and joint pain
- Fever
- Sweating
- Phase 2:
- Asthenia, characterized by:
- Muscle weakness
- Generalized fatigue
- Memory problems
- Difficulty concentrating
- Apathy
- Loss of appetite
- Localized pain, usually in the extremities
- Dizziness
- Lightheadedness
- Fainting
- Visual changes
- Shortness of breath
- Hypertension
- Diarrhea
- Blood in the stool
- Anemia: reduction in red blood cells
- Ischemia: decreased blood flow to tissues that can cause cellular stress (abnormal cell functioning)
- Decreased peripheral pulse, making it undetectable by palpation
Causes
The causes of Takayasu arteritis are currently unknown. However, studies suggest it is an autoimmune disease in which a genetic component predisposes the body to mistakenly attack itself. Despite this genetic predisposition, it is not considered an inherited disorder.
Risk Factors
Risk factors that increase the likelihood of developing Takayasu arteritis include:
- Sex: more common in women
- Age: usually manifests between 30 and 40 years of age, though it can also appear in childhood
- Genetic predisposition
Complications
The most frequent complications of Takayasu arteritis are:
- Stenosis: narrowing of blood vessels
- Arterial stiffening
- Aneurysm: dilation of arteries
- Hypertension
- Aortic tissue tearing
- Vessel obstruction causing reduced blood flow to vital organs:
- Stroke or cerebrovascular accident
- Myocardial infarction
- Heart inflammation
- Heart failure
- Aortic regurgitation: valve does not close properly, causing blood to flow back from the aorta into the heart
- Blurred vision
- Vision loss
- Retinopathy: inadequate blood supply to the retina causing microaneurysms and abnormal vessel connections (anastomoses)
Prevention
Takayasu arteritis cannot be prevented, as its causes remain unknown and it is an autoimmune condition.
Which Specialist Treats Takayasu Arteritis?
Takayasu arteritis is diagnosed by rheumatology specialists, who manage autoimmune diseases. Treatment may involve other specialties such as cardiology or angiology and vascular surgery.
Diagnosis
Diagnosis of Takayasu arteritis involves the following tests:
- Medical history: comprehensive collection of the patient’s lifestyle, overall health, medical and family history, and perceived symptoms
- Blood tests: provide relevant health information and detect signs of inflammation and anemia, a symptom of the disease
- Arterial auscultation: listening to blood vessel sounds with a stethoscope; abnormal sounds may indicate disease
- Angiography: a flexible catheter is inserted into an artery, usually the aorta, to obtain images. A contrast agent is injected, and X-rays are taken to observe blood flow, identifying narrowed or blocked areas
- Magnetic resonance imaging (MRI): a non-invasive procedure that visualizes blood vessels in detail. After contrast administration, radio waves and a magnetic field generate images without the need for catheter insertion
- Positron emission tomography (PET): measures the degree of vascular inflammation using a radioactive tracer visualized in bright colors on images obtained from multiple X-ray angles
Takayasu arteritis is confirmed when at least three of the following criteria are met:
- Patient age under 40 years
- Intermittent claudication: muscle pain, especially in the legs, that appears when walking and subsides at rest
- Reduced pulse in one or both brachial arteries (along the humerus)
- Systolic blood pressure difference >10 mmHg between arms
- Bruit over subclavian arteries or abdominal aorta: turbulent blood flow detected by auscultation
- Abnormal imaging results (stenosis or aneurysms)
Treatment
There is no definitive cure for Takayasu arteritis, but appropriate treatment allows disease control and symptom relief. The most effective approaches are:
- Medication: often required long-term and sometimes in combination to achieve desired effects
- Corticosteroids to control inflammation
- Immunosuppressants: reduce immune system activity to control inflammation and mitigate negative systemic effects; used in exceptional cases due to increased infection risk
- Anti-TNF monoclonal antibodies: biologic therapy that reduces inflammation, used when patients do not respond adequately to other medications
- Surgical treatment: necessary when arteries are severely damaged, limiting blood flow
- Angioplasty: a flexible tube with a balloon at its tip is inserted into the artery and inflated at the obstructed or narrowed site, guided by X-ray imaging
- Bypass surgery: a portion of a healthy blood vessel is placed alongside the obstructed area to create a detour for normal blood flow
