Takayasu arteritis

What is Takayasu's arteritis?

Takayasu’s arteritis is an inflammation of the blood vessels that affects the large blood vessels. The upper section of the aorta, including the aortic arch and the branches branching off from it to supply the brain and arms, are usually affected. The aorta transports blood – and therefore oxygen and nutrients – from the heart to the rest of the body. In Takayasu’s arteritis, the arterial wall becomes inflamed and thickened. This causes constrictions in the blood vessels, which in turn reduce blood flow. The vessel can even close completely. It is also possible for the affected vessel to dilate or bulge (aneurysm) if the vessel wall is weakened due to the inflammation. Such aneurysms are dangerous because they can rupture and cause bleeding.

Vascular inflammation is called vasculitis in medical terms and vasculitis in the plural. The name “Takayasu’s arteritis” goes back to the Japanese ophthalmologist Mikito Takayasu. The vascular disease is also known as pulseless disease because the pulses in the arms and legs often cannot be felt due to the narrowing of the corresponding vessels.

Takayasu arteritis – frequency and age

Takayasu’s arteritis is a very rare disease. Although the incidence of the disease varies in different countries, it can be roughly estimated as follows: 1 in 1 million inhabitants fall ill. This vasculitis is less common in Western Europe, but more frequent in Asia – especially in Japan, China, Thailand and India. It also occurs frequently in Africa and South America.

Takayasu arteritis mainly affects young women between the ages of 15 and 40. Women are affected three times as often as men.

Takayasu arteritis: causes are unclear

The causes of Takayasu arteritis are still largely unclear. However, it is suspected that a misdirected immune system plays an important role. In such autoimmune diseases, the immune system attacks the body’s own structures, for example the blood vessels. Genetic factors could also be involved. This is supported by the fact that the disease occurs more frequently in some families.

Symptoms: Takayasu's arteritis manifests itself in two phases

The symptoms of Takayasu’s arteritis depend on which vascular area is inflamed. The inflammation often affects:

• the first part of the aorta that comes out of the heart (aortic arch),
• the arm arteries (subclavian artery, axillary artery),
• the arteries supplying the brain (carotids).

However, the abdominal aorta and its vascular outlets such as the renal and intestinal arteries can also be affected. In some patients, the vascular inflammation can also spread from the aorta to the heart valves, so that the heart is also examined. Depending on the vessel affected, a wide variety of symptoms are possible. Takayasu’s arteritis usually progresses in two phases. Initially, vascular disease often manifests itself through general symptoms, which can also occur in the context of many other diseases. These include, for example:

Takayasu’s arteritis: symptoms of the first phase

• Tiredness, fatigue, exhaustion,
• Weakness, low physical performance, lack of endurance in arm or leg work,
• Increased body temperature, fever,
• unwanted weight loss,
• Night sweats,
• Muscle pain and joint pain.

Not all sufferers develop these symptoms, and some lack them altogether. It is therefore possible that the inflammation has been damaging the arteries for years without you noticing.

Phase two then follows after a few weeks and months. Many of those affected now experience symptoms that can be traced back to the circulatory disorders in the respective areas. They often affect larger parts of the body such as entire extremities or organs.

Takayasu’s arteritis: symptoms of the second phase

• The pulse in the arteries of the arm is weaker or the blood pressure in the arm is unusually low or can no longer be measured correctly.
• Pain in the hands, arms or legs (“PAD” or intermittent claudication) during movement and physical exertion
• Rarely, pain may also occur in the area of the carotid artery
• Pale and cool hands, especially when working
• Skin changes, for example inflammation of the subcutaneous fatty tissue (erythema nodosum) or hives (urticaria)
• Headaches, visual disturbances, visual field defects, thinking and concentration disorders, signs of paralysis, speech disorders if vessels supplying the brain are affected – they correspond to the symptoms of a stroke
• Back pain
• Symptoms of Raynaud’s phenomenon: The extremities suddenly turn pale in the cold or under stress. They turn bluish in color and some time later reddish when blood circulation resumes.
• Chest tightness(angina pectoris), shortness of breath and heart attack if coronary vessels are affected or a heart valve is affected

Diagnosis Takayasu arteritis

Initially, the symptoms of Takayasu’s arteritis are very unspecific. They can also indicate other diseases. Vascular inflammation is therefore usually only diagnosed when circulatory disorders occur. Takayasu’s arteritis is also a rare disease, which makes diagnosis even more difficult. The first step is always to discuss the patient’s medical history, the anamnesis. The following questions and answers provide us with initial clues:

• What symptoms have you noticed?
• How long have the symptoms been present and how severe are they?
• Do you experience situations in which the symptoms improve or worsen (e.g. during physical exertion)?
• Do you have any known illnesses?

This is usually followed by a physical examination. For example, a difference in blood pressure between the two halves of the body or a missing pulse in the arm may indicate Takayasu’s arteritis. The blood test also provides further information. Inflammatory values such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) are almost always elevated. Anemia can also be present and can be seen in the blood count.

Imaging examinations show the condition of the blood vessels. We use the following:

• Color-coded duplex sonography: Ultrasound examination that can be used to visualize the arteries. Inflammatory changes in the vessel wall can be recognized as thickenings or constrictions. Blood flow disorders can also be detected.
• MR angiography: You will be given a contrast agent (usually via a vein in your arm) and a magnetic resonance imaging (MRI) scan will be performed. In the MRI images, arteries throughout the body can be examined for inflammation.
• Angiography / digital subtraction angiography (DSA): With the help of a contrast agent and an X-ray examination, we can visualize your vessels, usually via a puncture in the inguinal artery under local anaesthetic. Irregularities in the vessel walls such as constrictions or aneurysms can be detected.
• Positron emission tomography (PET) or PET-CT (PET in combination with computer tomography, CT): This is a newer method for diagnosing inflammation of the vessel walls. It is particularly suitable for the aorta. You receive a special contrast agent via the vein, which accumulates in inflamed tissue. This allows the inflamed arteries and the disease activity in the body to be visualized.

In 1990, the American College of Rheumatology developed some diagnostic criteria for Takayasu’s arteritis that we can use as a guide. The disease is present if three or more of the following criteria are met:

• Onset of the disease before the age of 40
• “PAD” (intermittent claudication) of the upper or lower extremities – those affected stop more frequently due to circulatory disorders and pain or notice a loss of strength in the arms when doing more arm work
• Blood pressure difference of more than 10 mmHg between both arms
• Weakened pulse or pulselessness of the brachial artery (arterial blood vessel in the upper arm)
• Vascular sounds that can be heard with a stethoscope
• Pathologically altered angiogram of the large vessels of the extremities – without evidence of vascular calcification (arteriosclerosis) or fibromuscular dysplasia (a disease of the arteries in which the connective and muscle tissue in the vessel wall proliferates)

Takayasu arteritis: prevention, early detection, prognosis

The causes of Takayasu’s arteritis are still virtually unknown. There are therefore no specific measures you can take to prevent vascular inflammation. And you have no influence on the suspected parties involved – genes and autoimmune disease.

Early diagnosis is also difficult for those affected because Takayasu’s arteritis initially causes very general symptoms such as tiredness, fatigue or weight loss. They also occur in many other diseases. There are also no specific means of early detection in general practice. However, examination methods have now improved to such an extent that earlier diagnosis of vascular disease is possible. Always consult a doctor promptly if you experience any symptoms that seem unusual.

Course and prognosis of Takayasu’s arteritis

The prognosis of Takayasu’s arteritis has improved thanks to earlier diagnosis and new treatment options. However, vascular inflammation can lead to a number of complications during the course of the disease. Sometimes the heart valve on the aorta no longer closes properly over time (aortic valve insufficiency) or heart failure or a stroke occurs. People with Takayasu arteritis should have their disease regularly monitored and checked by us. This allows consequential damage to be recognized and treated in good time. But even with therapy, relapses are possible – then the vascular inflammation flares up again and the symptoms can return.

Possible complications of Takayasu’s arteritis

Vascular inflammation can be associated with a number of complications. These include, for example:

• Hardening and narrowing of blood vessels that restrict blood flow to the organs and tissues,
• High blood pressureusually as a result of reduced blood flow to the kidneys,
• Heart inflammation that affects the heart muscle or the heart valves,
• Heart failure due to high blood pressure, heart inflammation or heart valve insufficiency. In the case of aortic valve insufficiency, this valve no longer closes properly and blood flows back to the heart. Sometimes several of these complications occur at the same time.
• Strokewhen the blood flow in the arteries supplying the brain is restricted or completely blocked.
• Transient ischemic attack (TIA): A temporary circulatory disorder of the brain that causes symptoms similar to a stroke. However, unlike a stroke, a TIA does not leave any permanent damage.
• Aortic aneurysm, when the vessel walls are weakened, expand and form a small sac or balloon – the vessel can burst, which is life-threatening due to internal bleeding.
• Myocardial infarctionwhen the blood supply in the coronary arteries is restricted and the heart muscle is no longer supplied with sufficient oxygen and nutrients.

Takayasu's arteritis: treatment with anti-inflammatory drugs

Takayasu’s arteritis was long considered difficult to treat and many treatments were not sufficiently successful. That has since changed. If therapy is started in good time and is sufficiently intensive, vascular inflammation can usually be brought under control. Serious organ damage can often be avoided in this way.