Brief overview
- Description: Takayasu arteritis is a rare immune system disease in which the aorta and its major vessels become inflamed and narrowed over time.
- Causes: The exact cause of Takayasu arteritis is not yet known. A faulty reaction of the immune system causes the body’s own cells to attack the vessel walls.
- Prognosis: Takayasu disease is not curable yet. Untreated, the disease is usually fatal. With therapy, most patients are free of symptoms in the long term.
- Diagnosis: Discussion with the physician, physical examination (including blood tests, ultrasound, MRI, CT).
- Symptoms: Mostly fever, night sweats, weight loss and aching limbs. Circulatory disorders of the arms and legs, dizziness, fainting, strokes or high blood pressure are also possible in the further course.
Takayasu arteritis (also Takayasu disease or Takayasu syndrome) is a rare autoimmune disease in which the walls of blood vessels become inflamed (vasculitis). The disease is named after the Japanese physician Mikado Takayasu, who first described the disease in 2008.
Takayasu syndrome belongs to the group of so-called primary vasculitides. This collective term refers to inflammatory diseases of the blood vessels for which there is no known underlying disease. Vasculitides belong to the rheumatic diseases because they are often accompanied by pain in the joints or muscles and sometimes also by joint swelling.
Who is affected?
Takayasu syndrome usually begins between the ages of 20 and 30. After the age of 40, the disease occurs rather rarely.
How does Takayasu’s arteritis develop?
The cause of Takayasu’s arteritis is not yet known. It is thought that a hereditary predisposition in combination with an unknown trigger (e.g. environmental factors such as radiation, toxins, stress, hepatitis viruses, infection with the bacterium Staphylococcus aureus) trigger the disease.
As a result, the organs and limbs are no longer supplied with sufficient oxygen. Depending on which vessels are affected, Takayasu disease results in different symptoms.
How is Takayasu arteritis treated?
As a result, the organs and limbs are no longer supplied with sufficient oxygen. Depending on which vessels are affected, Takayasu disease results in different symptoms.
How is Takayasu arteritis treated?
Immunosuppressants
If the affected person cannot tolerate the cortisone or the cortisone is not sufficient for treatment, the doctor prescribes other immunosuppressants such as methotrexate or cyclophosphamide as an alternative. These substances inhibit the body’s immune defenses and prevent the inflammation from progressing further in Takayasu’s arteritis. They have a very strong effect and are also used in the treatment of cancer (cytostatics).
Blood thinner
Antibody therapy
If the affected person does not respond to therapy with immunosuppressants, the physician may treat with a so-called TNF alpha blocker. These active substances belong to the group of biologics, genetically engineered drugs (e.g. antibodies). They are specifically directed against certain messenger substances that cause inflammation in the vessel walls.
Operation
Various surgical and minimally invasive procedures are available to make the blood vessels permeable again. These include balloon dilation, insertion of a stent or bypass surgery.
Balloon dilation
Stent
To stabilize the vessel and keep it open, in some cases the doctor inserts a stent (wire tube made of metal or plastic) after balloon dilation. To do this, the doctor pushes a catheter with the stent into the affected vessel via the guide wire and places it there. Once the stent has been placed, it remains in this form in the blood vessel and ensures that the blood flows freely again at the former constriction.
Bypass surgery
Serious complications of Takayasu arteritis, such as a heart attack or stroke, are largely preventable with proper therapy.
Is Takayasu arteritis curable?
According to a major study from Japan, the disease does not deteriorate further in two-thirds of those affected with the right therapy. Serious complications (e.g. heart attack, stroke) occur in only one quarter.
The earlier the doctor recognizes and treats the disease, the lower the risk of permanent damage.
How does the doctor make the diagnosis?
Since the symptoms of Takayasu’s arteritis are usually quite unspecific at the beginning of the disease and the disease itself is very rare, it is important for the doctor to examine the affected person in detail. To this end, the physician first conducts a detailed interview (anamnesis) with the affected person. He then conducts a physical examination of the patient.
Conversation with the doctor
During the conversation, the doctor asks the affected person, among other things:
- When did the complaints occur?
- Are there any pre-existing conditions (e.g. rheumatism, arteriosclerosis, high blood pressure)?
- What are your lifestyle habits? Do you exercise regularly? What is your diet like? Do you smoke?
Physical examination
Ultrasound
During an ultrasound examination of the blood vessels (color duplex sonography), the physician sees whether the vessel wall is constricted or inflamed. He also uses ultrasound to determine the direction in which the blood flows in the vessels and how fast the blood flows through the vessels (flow velocity). The latter provides the physician with information on whether there are constrictions or occlusions in the vessels.
In some cases, the doctor inserts an ultrasound probe into the esophagus (transesophageal echocardiography, TEE) to examine the vessels that are close to the heart.
Angiography
Blood test
The physician also examines the blood of the affected person. If, for example, the erythrocyte sedimentation rate (ESR) is greatly increased, this is an indication of an inflammatory disease such as Takayasu’s arteritis. The ESR indicates how quickly the red blood cells in a blood sample sink within an hour in a special tube.
ACR criteria
Based on the results of all examinations, the physician makes the diagnosis. This is usually done on the basis of the so-called ACR criteria (ACR stands for American College of Rheumatology). If at least three of the criteria are met, it is most likely Takayasu’s arteritis:
- The affected person is under 40 years of age.
- There are circulatory disturbances in the arms and/or legs, e.g. limping (claudication) or muscle pain when the affected person moves.
- The systolic blood pressure between the two arms differs by more than 10 mmHg (systolic = the blood pressure when the heart muscle contracts and pumps oxygen-rich blood into the vessels).
- Flow sounds of blood over the aorta or the artery under the clavicle (subclavian artery) can be heard with a stethoscope.
- Changes in the arteriogram (e.g. vascular changes in the aorta) can be detected.
What are the symptoms?
With the onset of Takayasu’s arteritis, general and non-specific inflammatory reactions of the body usually occur. Affected individuals usually feel very ill. They are exhausted and weak, have no appetite, and report joint and muscle pain. The most common symptoms are:
- Fatigue
- Mild fever (about 38 degrees Celsius).
- Unwanted weight loss
- Night sweats
- Loss of appetite
- Weakness
- Muscle and joint pain
If the disease is already advanced, chronic complaints develop. These develop because the vessels narrow over time and no longer supply the organs and limbs with sufficient blood. The symptoms that develop depend on the artery affected. The most common symptoms of advanced Takayasu arteritis include:
- Circulatory problems in the arms and/or legs
- Dizziness (vertigo)
- Fainting spells
- Visual disturbances
- Stroke (cerebral insult)
- Heart attack (myocardial infarction)
- Aneurysm (balloon-like bulging of the vessels)
Circulatory disorders
In principle, it is possible for Takayasu’s arteritis to involve the entire aorta and all its side branches from the heart to the groin. Most often, however, the arms are damaged, secondarily the arteries supplying blood to the brain.
Dizziness and fainting spells
In addition, the pulse at the wrist is often weakened or cannot be felt at all. Blood pressure is often different in the two arms. If the arteries in the carotid artery are affected, people with Takayasu syndrome often also have pain in the side of the neck.
High blood pressure
Strokes and heart attacks
If the vessels are inflamed, there is also a risk that they will narrow considerably over time and even close completely (arterial stenosis). The affected areas are then no longer supplied with sufficient oxygen and nutrients, and the tissue in this area dies. As a result, strokes (caused by narrowed vessels in the brain) or heart attacks (caused by narrowed vessels in the heart muscle) often occur in untreated Takayasu arteritis.
Since there is high blood pressure in the vessels near the heart, the vessels expand increasingly when the vessel walls are weakened as a result of the inflammation. Sometimes this results in the formation of bulges in the vessel wall (aneurysms). If such an aneurysm ruptures, life-threatening internal bleeding usually results. The dangerous thing about these dilated vessels is that they usually do not cause any symptoms.
More symptoms
Seek medical attention immediately if you find it difficult to breathe and have chest or arm pain!
